about the con fer ence - Neurologijos seminarai

Transcription

s e m i n a r a i
N e u r o l o g i j o s
ISSN 1392-3064
LIETUVOS NEUROLOGØ ASOCIACIJA
LIETUVOS VAIKØ NEUROLOGØ ASOCIACIJA
LIETUVOS NEUROCHIRURGØ DRAUGIJA
9TH INTERNATIONAL CONFERENCE OF BALTIC
CHILD NEUROLOGY ASSOCIATION (BCNA)
JUNE 20–23, 2007
VILNIUS, LITHUANIA
FINAL PROGRAM AND ABSTRACT BOOK
9-OJI TARPTAUTINË BALTIJOS ÐALIØ
VAIKØ NEUROLOGØ ASOCIACIJOS KONFERENCIJA
2007 M. BIRÞELIO 20–23 D.,
VILNIUS, LIETUVA
PROGRAMA IR TEZËS
2007, T. 11, Priedas Nr. 1
VILNIUS „ROTAS“
2007
NEUROLOGIJOS SEMINARAI
SEMINARS IN NEUROLOGY
Leidþiamas nuo 1997 m.
Steigëjas: VU MF Neurologijos klinika
VYRIAUSIASIS REDAKTORIUS • EDITOR-IN-CHIEF
Valmantas BUDRYS (Vilniaus universitetas)
ATSAKINGOJI REDAKTORË • EXECUTIVE EDITOR
Daiva RASTENYTË (Kauno medicinos universitetas)
REDAKTORIØ KOLEGIJA • EDITORIAL BOARD
Ilona BIÈKUVIENË (Vilniaus universitetas)
Algirdas DEMBINSKAS (Vilniaus universitetas)
Milda ENDZINIENË (Kauno medicinos universitetas)
Egidijus JARÞEMSKAS (Vilniaus universitetas)
Dalius JATUÞIS (Vilniaus universitetas)
Alvydas JUOCEVIÈIUS (Vilniaus universitetas)
Romas A. GVAZDAITIS (Kauno medicinos universitetas)
Gintaras KAUBRYS (Vilniaus universitetas)
Michel R. MAGISTRIS (Geneva, Switzerland)
Diana OBELIENIENË (Kauno medicinos universitetas)
Regina PARNARAUSKIENË (Vilniaus universitetas)
Valius PAUZA (Kauno medicinos universitetas)
Audrius V. PLIOPLYS (Chichago, IL, USA)
Arûnas ÐÈIUPOKAS (Kauno medicinos universitetas)
Juozas ÐIDIÐKIS (Kauno medicinos universitetas)
Arimantas TAMAÐAUSKAS (Kauno medicinos universitetas)
Nerija VAIÈIENË (Kauno medicinos universitetas)
Reinhard VOLKMANN (Göteborg, Sweden)
Walter-Uwe WEITBRECHT (Gummersbach, Germany)
Redaktoriø kolegijos adresas:
Vilniaus universiteto ligoninës Santariðkiø klinikos
Santariðkiø g. 2, LT-08661 Vilnius
Tel./faks. (8 5) 236 52 20
El. p.: valma@takas.lt
http://www.neuro.lt
Editorial address:
Vilnius University Santariðkiø Klinikos Hospital
Santariðkiø 2, LT-08661 Vilnius, Lithuania
Tel./fax (370 5) 236 52 20
E-mail: valma@takas.lt
http://www.neuro.lt
Leidinyje pateikiama informacija skirta tik specialistams medikams.
Redakcijos nuomonë nebûtinai sutampa su straipsniø autoriø nuomone.
Redakcija uþ reklamos turiná ir kalbà neatsako.
Visi þurnale minimi vaistai turi bûti vartojami, atsiþvelgiant á naujausià vaistø vartojimo informacijà.
„Neurologijos seminarø“ leidybinës teisës yra leidëjo nuosavybë, saugoma autorinës teisës. Visos ar bet kurios
þurnale spausdinamos medþiagos dalies dauginimui ir platinimui bet kokia forma ir priemonëmis ne asmeniniams
tikslams bûtinas raðtiðkas leidëjo sutikimas.
„Neurologijos seminarai“ átraukti á Index Copernicus duomenø bazæ, kurioje referuojamø leidiniø moksliniai
straipsniai pripaþástami tinkamais vertinant mokslo darbuotojø, kitø tyrëjø ir dëstytojø kvalifikacijà (Lietuvos
mokslo tarybos 2006 m. balandþio 24 d. nutarimas Nr. VI-30)
Kalbos redaktorë J. Niaurienë
Maketavo V. Viluèio ámonë
2007 06 07. 6 spaudos lankai
SL 021. Uþs. 208
Spausdino spaustuvë „Rotas“, Pylimo g. 42, LT-01136 Vilnius
© Neurologijos seminarai, 2007
TABLE OF CONTENTS
Patronage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
Welcome of the Organizing Committee . . . . . . . . . . . . . . 5
Conference Organization . . . . . . . . . . . . . . . . . . . . . 7
Sponsors and Exhibitors . . . . . . . . . . . . . . . . . . . . . . 8
About the Conference . . . . . . . . . . . . . . . . . . . . . . . 9
Social Program . . . . . . . . . . . . . . . . . . . . . . . . . . 11
General Information. . . . . . . . . . . . . . . . . . . . . . . . 12
Scientific Program June 21 . . . . . . . . . . . . . . . . . . . . 13
Abstract Book . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Oral Presentations. . . . . . . . . . . . . . . . . . . . . . 18
Poster Presentations . . . . . . . . . . . . . . . . . . . . 30
Index of Authors . . . . . . . . . . . . . . . . . . . . . . . . . 41
List of Participants . . . . . . . . . . . . . . . . . . . . . . . . 45
Mrs. Alma Adamkienë, First Lady of the
Republic of Lithuania is the patroness of the
9th International Conference of Baltic Child
Neurology Association.
DEAR FRIENDS AND COLLEAGUES,
It is a great pleasure to welcome you at the 9th International Conference of Baltic Child
Neurology Association (BCNA) in Vilnius on June 20-23, 2007. BCNA Conferences are
held every other year and have turned out to be not just regional but also international forums for professionals interested in the improvement and development of care for children with neurological diseases. We are happy to declare the program of the 9th BCNA
Conference which includes not only the usual “hot” topics of pediatric neurology like epilepsy or developmental disorders, but much attention is given to neurogenetic and
neurometabolic diseases, also to the relatively new concept in our region – Orphan diseases and Orphan drugs. Interdisciplinary aspects of pediatric neurology will also be covered, including neuropsychiatry, or questions related both to pediatric neurology and neurosurgery like tumors, trauma, and hydrocephalus. A special lecture for young researches
on preparation of publications is most valuable. Poster sessions will provide the opportunity of lively discussions with experts and followed by the nomination of the best poster.
The geography of our delegates has expanded to 18 countries; the number of registered
participants has exceeded 200. We are proud to have so many outstanding speakers with
updated lectures and are happy to welcome all our colleagues and friends who are going
to share their personal professional experience, both in formal and informal ways.
Vilnius, the historical capital of Lithuania dating back to the 14th century, has the most
beautiful and the largest Old Town, awarded with the status of World Cultural Heritage by
UNESCO, with Vilnius University being the oldest one in Eastern Europe. It is rapidly expanding as a modern European capital, so please use the chance of experiencing the harmony of the old and the new Vilnius. For all of us this will provide beautiful atmosphere and
the mood for work and friendship.
Enjoy your stay in Lithuania
Sveiki atvykæ! Welcome!
With warmest regards,
Nerija Vaièienë
Conference President
Milda Endzinienë
Conference Vice-President
A high fat, low carbohydrate, nutritionally
complete, powdered formula for the
dietary management of drug resistant
epilepsy in children over the age of
one year.
Formulated to induce and maintain
ketosis. It is based on the classical
4:1 ratio (4g fat: 1g protein and
carbohydrate).
Convenient and easy to prepare as it is
nutritionally complete - just measure
and shake.
Administer as a tube or sip feed either
as a sole source of nutrition or meal
replacement in a choice of flavoured
and unflavoured 300g cans.
Unflavoured
Vanilla Flavoured
Simplifies Initiation
and Delivery of the
Ketogenic Diet
For more information please contact:
info@nutricia.lt
SHS International office in Baltic countries: UAB Nutricia Baltics, Zirmunu str. 68, 124-Vilnius, Lithuania. Phone: (+370 5) 23 00 377 Fax: (+370 5) 23 00 404
CONFERENCE ORGANIZATION
Main organizers:
Lithuanian Child Neurology Association
Ministry of Health of the Republic of Lithuania
Conference President
Professor Nerija Vaièienë
Ex-president and vice-president of Lithuanian Child Neurology Society
President of Lithuanian Chapter of ILAE
Department of Neurology
Kaunas University of Medicine
Eiveniu str. 2, LT-50009 Kaunas, Lithuania
Tel. +370 37 326213
Fax. +370 37 326922
E-mail: nerija.vaiciene@kmu.lt
Conference Vice-President
Professor Milda Endzinienë
President of Lithuanian Child Neurology Society
Ex-president and vice-president of Lithuanian Chapter of ILAE
Department of Neurology
Kaunas University of Medicine
Eiveniu str. 2, LT-50009 Kaunas, Lithuania
Tel. +370 37 326811
Fax. +370 37 326 852
E-mail: endziniene@gmail.com
Scientific Committee
Nerija Vaièienë (Lithuania)
Milda Endzinienë (Lithuania)
Egils Vitols (Latvia)
Tiina Talvik (Estonia)
Local Organizing Committee
Jûratë Grigonienë
Rûta Praninskienë
Tatjana Volkova
Rûta Samaitienë
Edita Kanytë
Daiva Stankevièiûtë
Conference Secretariat
Conbaltas UAB
Baltic Conference Organizers – PCO
Kareiviu str. 6, LT-09117 Vilnius, Lithuania
Tel. +370 5 2120003
Fax +370 5 2120013
E-mail: bcna@bcna2007.com
Website: www.bcna2007.com
S7
ACKNOWLEDGEMENT
Organizing Committee of the 9th International Conference of Baltic Child
Neurology Association is thankful for contribution of:
Gold Sponsor
Sponsors and Exhibitors
S8
9th International Conference of Baltic Child Neurology Association (BCNA) • June 20–23, 2007, Vilnius, Lithuania
ABOUT THE CONFERENCE
Conference Dates
June 20-23, 2007
Conference Venue
The Conference will take place in Reval Hotel
Lietuva. It is located on the beautiful bank of the
river Neris in the center of Vilnius.
Reval Hotel Lietuva
Konstitucijos Ave. 20
Vilnius LT-09308, Lithuania
Official Language
English. No simultaneous translation will be
provided.
On-Site Registration Fees
On site, in Euro
Participant
200
Resident and Student
70
Accompanying Persons
40
Gala Dinner
60
One Day Participant Fee
70
One Day Resident Fee
25
Welcome Reception*
30
* – charge is applied only for one day delegates.
Registration Fee includes:
• Attendance to all scientific sessions and the
exhibition
• Scientific documentation and a Conference bag
• Welcome Reception
• Coffee and lunches during the Conference
• Certificate of attendance
Accompanying Person’s Registration Fee
includes:
• Welcome Reception
• Vilnius City Tour
One Day Registration Fee includes:
• Attendance to that day scientific sessions
• Scientific documentation and Conference bag
• Coffee breaks and lunch during the Conference
• Certificate of attendance
Payment
Payments on site should be made either in cash
(Euro or Litas) or by credit card. Only registered
and paid delegates can attend the Conference.
Registration and Hospitality Desks
The Registration and Hospitality Desks for the
Conference will be located in the lobby of the
Conference Center of Reval Hotel Lietuva. They will
be open on:
Wednesday
Thursday
Friday
Saturday
June 20, 15:00 – 18:30
June 21, 08:00 – 19:00
June 22, 08:30 – 18:00
June 23, 08:30 – 13:00
Conference Identification Badges
The Conference Identification Badge will be
included in the Conference material provided while
registering for the Conference. There will be no
admittance to the scientific sessions without the
Conference Badge. Tickets to the social events will
be collected on entry. The Conference
Identification Badges will be also most helpful in
contacts with other participants. Cost of replacing
a lost or mislaid badge: 10 Euro.
Certificate of Attendance
Certificates of Attendance will be distributed on
June 23.
Abstract Book
All the abstracts that have been accepted by the
Scientific Committee are published in the scientific
journal “Neurologijos Seminarai”. Companies and
delegates may copy materials for their personal
use, but further copying for sale or for any other
commercial purpose is prohibited without prior
written permission by the Editor.
Speakers’ Room
The speakers will be able to check their
presentations in advance in the Speakers’ Room
which will be located in Epsilon hall. It will be open
on:
Thursday
Friday
Saturday
June 21, 08:00 – 19:00
June 22, 08:30 – 18:00
June 23, 08:30 – 12:00
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The Speakers’ Room is equipped with computers.
Please note that the presentations should be
downloaded in the Speakers’ Room. All the
speakers are kindly requested to provide the
electronic versions of their presentations one
day prior to the session (especially important
for video presentations).
Exhibition
A commercial and technical exhibition will form an
integrated part of the Conference. It will take place
in the lobby of the Conference Center of Reval
Hotel Lietuva.
Opening Hours:
Thursday
Friday
Saturday
June 21, 08:00 – 19:00
June 22, 08:30 – 18:00
June 23, 08:30 – 12:00
Poster Exhibition
The Poster Exhibition will take place in the lobby of
the Conference Center of Reval Hotel Lietuva.
The Poster Exhibition will open on:
Thursday
June 21, 08:00 – 19:00
Friday
June 22, 08:30 – 18:00
Saturday
June 23, 08:30 – 12:00
S10
The posters should be brought in person and put
up on
Wednesday
June 20, 15:00 – 18:30
The posters should be taken off
Saturday
June 23, 13:00 – 14:00
Posters that will not be taken off by the specified
deadline will be destroyed.
During the Poster Exhibition on 22 of June the
best poster will be selected and awarded!
Internet Corner
The Internet Corner will be located in the lobby of
the Conference Center of Reval Hotel Lietuva. It is
free of charge and it will be open during all
Conference hours.
Coffee Breaks
Coffee, tea and other refreshments will be served in
the exhibition area as well as in the lobby of the
Conference Center of Reval Hotel Lietuva.
Lunches
Lunches will be served in restaurant “River Side” at
the Reval Hotel Lietuva.
SOCIAL PROGRAM
THE WELCOME RECEPTION
June 20, Wednesday. 19:00
Free for participants and accompanying persons*
VILNIUS CITY TOUR
June 22 Friday. 13:00
Free for accompanying persons
A Welcome Reception followed by tender music
will take place in the Reval Hotel Lietuva, Beta
Hall – Conference venue, (Konstitucijos Ave 20),
situated on the right bank of the river Neris, in the
center of Vilnius. Luscious food and refreshing
drinks will set everyone for a pleasant evening. This
is perfect time to meet old colleagues and make
new contacts.
Dress code – smart casual.
*Entrance fee for One Day Participants is 30 Euro.
Vilnius City Tour continues for 2.5 hours (on foot)
and takes you through the most interesting places
in Vilnius Old Town. Old Town is the largest in
Eastern Europe. Vilnius has always attracted
visitors and fascinated everyone with its
architectural diversity. The ruins of castles, an old
network of narrow streets, church spires, bell
towers, red tile roofs, residential cellars...
GALA DINNER AT BELMONTAS
ENTERTAINMENT CENTER
June 22, Friday. 20:00
Entrance fee is 60 Euro
Excursion starts at 13:00 at the Cathedral Square.
The tour is included in the registration fee of
accompanying persons (please pick up your
tickets at the Hospitality Desk); for others price
per person is 15 Euro.
A Gala Dinner will take place in the remarkable
Belmontas Entertainment Center, at the
restaurant – mill of the Carl de Vimo, located in the
picturesque surroundings of Vilnius. Romantic
pedestrians path will give you pleasure for a walk
in the fresh air by the river under the starlight sky.
Musical orchestra “Mezzo” will play unforgettable
Frank Sinatra and Elvis Presley time music together
with outstanding Lithuanian soprano Auðra
Liutkutë. Comfortable busses will pick you up
and bring to the restaurant. Delicious food and
tender music will treat everyone for the beautiful
moments.
Dress code – smart casual.
S11
GENERAL INFORMATION
About Lithuania
Lithuania is the largest and southernmost of the
Baltic States. Lithuania is bordered by Latvia to the
north, Belarus to the east and south and Poland
and Russian Federation to the south and
southwest. Forest spread through almost
30 percent of the country, and rivers, streams and
more than 3000 lakes shape the land.
Five national and 30 regional parks preserve this
paradise for boating, fishing, hunting, bird
watching or horseback riding. The largest river,
Nemunas, flows into the Curonian Lagoon
separated from the Baltic Sea by the Curonian Spit.
This 98 km long bank of sand, dunes and pine
trees stretches from Kalingrad in the south to the
seaport of Klaipeda in the north. The Curonian Spit
recognized by UNESCO as a part of the world
heritage, is one of the most unspoiled natural spots
in the whole Baltic Region. It is also known as the
amber coast, for the precious amber that was
washed onto this shore for many centuries.
About Vilnius
The capital, Vilnius, is one of the most beautiful
towns in Eastern Europe, situated where the Neris
and Vilnele rivers meet and surrounded by
picturesque wooded hills. Founded in 1323, its
unique character and architecture reflects its history
at crossroads of the Roman, Byzantine and
Eurasian worlds. Vilnius’ many churches and
towers, fortifications, secluded medieval courtyards
and narrow streets are a mix of Gothic,
Renaissance, and Baroque and Classical styles.
Vilnius, like all Lithuanian cities, has a wide selection
of restaurants. Lithuanian beer is widely considered
to be some of the best in the world. Drink it with
tasty local breads and cheeses or national dishes
like “zeppelins”, balloon-shaped potato cakes filled
with meat, or potato sausages and pancakes.
In 2009 Lithuania will celebrate a full millennium since
the first mentioning of its name in historical sources.
Climate
Lithuania has a central European climate. The end
of June generally is warm. Take a sweater for a
cool evening and an umbrella for a shower.
Time
Eastern Europe Summer time 3 hours ahead of
Greenwich Mean Time.
Bus Services
Vilnius has an effective and cheap network of
busses and minibuses. Prices for buses and
minibuses are up to 1 Euro.
Taxi
Numerous taxis are operating in Vilnius. Taxis hired
in street can be expensive and not always of the
best quality. It is recommended to call Martono Taxi
1422 or +37052400004. The price for a taxi from
the airport to the center is around 15 Euros.
S12
PROGRAM
20 June, Wednesday
15:00 – 18:30 Registration
19:00
Welcome Reception
21 June, Thursday
8:30 – 9:20
Opening of the Conference
9:20 – 11:00
Morning session I: PERINATAL NEUROLOGY AND DISABILITY
Chairing: Tiina Talvik (Estonia), Eugen Boltshauser (Switzerland)
9:20 – 9:50
Brigitte Vollmer (Sweden)
“Follow up of preterm children: relationship between brain structure
and clinical outcome”
9:50 – 10:20 Michel Magistris (Switzerland)
“Obstetric brachial plexus palsy: old problem, new understanding”
10:20 – 10:45 Anita Vetra (Latvia)
“Spasticity in children”
10:45 – 11:00 Laima Mikulënaitë, Rûta Samaitienë, Liuda Istigeèeva (Lithuania)
“Improvement of functional abilities in children with cerebral palsy
treated with botulinum toxin”
11:00 – 11:30 Coffee break
11:30 – 13:00 Morning session II: BETWEEN NEUROLOGY AND NEUROSURGERY
Chairing: Egils Vitols (Latvia), Vytautas Ragaiðis (Lithuania)
11:30 – 12:00 Liana Beni-Adani (Israel)
“The occurrence of obstructive versus absorptive hydrocephalus in
newborns and infants: relevance to treatment choices”
12:00 – 12:15 Dovilë Grinkevièiutë, Vytautas Ragaiðis (Lithuania)
“Intracranial pressure-targeted treatment of severe pediatric head
injury: Lithuanian experience”
12:15 – 12:30 Inga Talvik, Mairi Mannamaa, Tiina Talvik (Estonia)
“Inflicted traumatic brain injury or shaken baby syndrome in Estonia:
incidence and long-term outcome”
12:30 – 12:45 Rosita Kiudelienë, Giedrë Rutkauskienë, Algimantas Matukevièius,
Donata Jurgaitytë, Lingvita Gumbelevièienë, Nerija Vaièienë (Lithuania)
“Pediatric brain tumors in Lithuania: epidemiological overview”
12:45 – 13:00 Algimantas Matukevièius (Lithuania)
“Brainstem gliomas in children”
S13
13:00 – 14:00 Lunch
14:00 – 16:00 Afternoon session I: EPILEPSY I
Chairing: Rochelle Caplan (USA), Ulrich Stephani (Germany)
14:00 – 14:30 Olivier Dulac (France)
“Metabolic diseases with epilepsy”
14:30 – 15:00 Marina Nikanorova (Denmark)
“Epilepsies of childhood: diagnostic challenges”
15:00 – 15:30 Walter Van Emde Boas (The Netherlands)
“Frontal lobe epilepsies in childhood: semiology and clinical spectrum”
15:30 – 15:45 Nebojsa Jovic (Serbia)
“Malformations of cortical development: is prognosis of epilepsy
predictable?”
15:45 – 16:00 Jurgis Strautmanis (Latvia)
“Should we treat epilepsy?”
16:30 – 18:30 Afternoon session II: NEUROPSYCHIATRY AND COGNITIVE DISORDERS
Chairing: Lene Sahlholdt (Denmark), John Stephenson (Scotland)
16:30 – 17:00 Sigita Plioplys (USA)
“Current views on pediatric psychogenic non-epileptic seizures”
17:00 – 17:30 Rochelle Caplan (USA)
“Diagnosis and feedback in pediatric non-epileptic seizures: do’s and don’ts”
17:30 – 17:50 Aurelija Juèaitë (Sweden)
“Attention deficit hyperactivity disorder: alterations of dopaminergic system”
17:50 – 18:10 Darius Leskauskas (Lithuania)
“Prevalence and current treatment of attention deficit – hyperactivity
disorder in Lithuania primary school pupils”
18:10 – 18:30 Anneli Kolk (Estonia)
“Cognitive dysfunction of children with symptomatic and newly
diagnosed epilepsy”
22 June, Friday
8:30 – 10:30
Morning session I: EPILEPSY II
Chairing: Nerija Vaièienë (Lithuania), Walter van Emde Boas (The Netherlands)
8:30 – 9:00
Perrine Plouin (France)
“Seizure semiology and syndromic approach in children under 3 years”
9:00 – 9:30
Athanasios Covanis (Greece)
“Panayiotopoulos syndrome vs Gastaut-type occipital epilepsy”
9:30 – 10:00 Rochelle Caplan (USA)
“Psychiatric and cognitive comorbidities in pediatric epilepsy: cause or
effect?”
10:00 – 10:30 Sigita Plioplys (USA)
“Pharmacological treatment of attention deficit hyperactivity disorder in
children with epilepsy”
S14
11:00 – 13:00 Morning session II: GENETIC AND DEGENERATIVE DISORDERS
Chairing: Birutë Skerlienë (Lithuania), Leo Spaapen (The Netherlands)
11.00 – 11:30
Ulrich Stephani, Hiltrud Muhle, Sarah von Spiczak (Germany)
“Current achievements in the genetics of the epilepsies”
11:30 – 12:00 Eugen Boltshauser (Switzerland)
“Cerebellar atrophies”
12:00 – 12:30 Lina Basel - Vanagaitë (Israel)
“Genetics of mental retardation: present and future”
12:30 – 13:00 Thomas Sejersen (Sweden)
“Future treatments of neuromuscular disorders”
13:00 – 14:00 Lunch
14:00 – 15:30 Afternoon session I: RARE DISEASES AND ORPHAN DRUGS
Chairing: Milda Endzinienë (Lithuania), Thomas Sejersen (Sweden)
14:00 – 14:25 Leo Spaapen (The Netherlands)
“Inherited metabolic causes of neurological diseases in neonates and
infants”
14:25 – 14:50 Arrigo Schieppatti (Italy)
“Orphan drugs and rare diseases: problems and opportunities”
14:50 – 15:15 Paola Baiardi, Manuel de La Paz, Lars Savendahl, Domenica Teruscio,
Renza Barbon Galluppi, Enrico Morten, Donatas Stakisaitis,
Adriana Ceci (Italy)
“Accessing information on orphan drugs: the EuOrphan Service”
15:15 – 15:30 Indrë Ðpokienë (Lithuania)
“Orphan patients and orphan medicines: situation in Lithuania”
16:00 – 16:45 SPECIAL TOPIC!
Roger A. Brumback (USA)
“Publishing in biomedical journals: hints for success from a journal Editor”
16:45 – 17:30 POSTER SESSION
Moderators: Marina Nikanorova (Denmark), Michel R. Magistris (Switzerland),
Athanasios Covanis (Greece)
20:00
Gala Dinner
S15
23 June, Saturday
9:00 – 10:30
Morning session I: EPILEPSY AND DISABILITY
Chairing: Sigita Plioplys (USA), Nebojsa Jovic (Serbia)
9:00 – 9:15
Jurgita Grikinienë (Lithuania)
“Epilepsy, magnesium and gender differences”
9:15 – 9:30
Ljerka Cvitanovic Sojat, Masa Malenica, Romana Gjergja, Zlatko Sabol,
Tina Sojat (Croatia)
“Febrile seizures and epilepsy: a retrospective study”
9:30 – 9:45
Ulvi Vaher, Aita Napa, Tiina Talvik (Estonia)
“Seizures and value of EEG investigations in asphyxiated term newborn
infants”
9:45 – 10:00 Jûratë Grigonienë, Dovilë Raèkauskaitë, Milda Endzinienë,
Nerija Vaièienë (Lithuania)
“Neurodevelopmental outcome of neonatal seizures”
10:00 – 10:15 Svajûnë Gradeckienë, Danguolë Ostrauskienë, Lina Norkienë,
Asta Kutraitë, Milda Endzinienë, Nerija Vaièienë (Lithuania)
“Treatment of spasticity with botulinum toxin A in cerebral palsy: three
years experience at Kaunas University Hospital”
10:15 – 10:30 Ugur Cavlak, Naile Guney (Turkey)
“Physical characteristics of unaffected limbs of cerebral palsied children”
11:00 – 12:30 Morning session II: MISCELLANEOUS
Chairing: Guntis Rozentals (Latvia), Aurelija Juèaitë (Sweden)
11:00 – 11:15 Birutë Skerlienë, Jûra Tulevièienë, Marija Jakutoviè, Donatas Petroðka
(Lithuania)
“Muscular presentation of inherited disorders of mitochondrial
metabolism in children”
11:15 – 11:30 Nerija Vaièienë (Lithuania)
“Juvenile onset Huntington disease”
11:30 – 11:45 Milda Endziniene (Lithuania)
“A case of severe Pelizaeus-Merzbacher disease caused by the
triplication of PLP1 gene”
11:45 – 12:00 Rûta Praninskienë, Aurelija Juèaitë, Irena Dumalakienë,
Mykolas Mauricas (Lithuania)
“Melatonin secretion in children: developmental and neurological
disorders”
12:00 – 12:15 Rûta Samaitienë, Laima Mikulënaitë, Liuda Istigeèeva (Lithuania)
“Sleep disorders in children with developmental disabilities”
12:15 – 12:30 Karlis Kupcs, Helmuts Kidikas, Igors Aksiks, Svetlana Rudnicka,
Aleksejs Vinogradovs, Janis Savlovskis (Latvia)
“Endovascular treatment of intracranial aneurysms in children – first
experience”
12:30
S16
Closing of the Conference
ABSTRACT BOOK
ORAL PRESENTATIONS
21 June, Thursday
FOLLOW-UP OF PRETERM CHILDREN:
RELATIONSHIPS BETWEEN BRAIN
STRUCTURE AND CLINICAL OUTCOME
Brigitte Vollmer
Karolinska Institute, Stockhol, Sweden
Infants born prematurely are at high risk of long term neurological and neurodevelopmental impairments. About 10–15%
of those born at < 32 weeks of gestation will have major impairments such as cerebral palsy, severe visual impairment,
global cognitive delay and/or epilepsy. Brain lesions in
preterm children are typically located in the periventricular
white matter. It has been shown that there is a relationship between location and extent of these lesions as seen on conventional structural magnetic resonance imaging (MRI) and outcome as far as the major impairments are concerned. Up to
30% of those preterms who survive without major impairments will have minor neurological signs, specific cognitive
deficits, attentional and behavioural difficulties and these minor impairments have been shown to have significant implications for e.g. educational progress of children born prematurely. It has been suggested that these deficits are partly
caused by subtle structural brain abnormalities that may be
too subtle to be identified by purely visual analysis of MR images. Indeed results from recent studies that have used sophisticated MRI acquisition and analysis techniques indicate
that subtle structural brain abnormalities are associated with
some of the minor difficulties seen in children born preterm. In
this presentation I will discuss both the relationships between
major impairments and brain abnormalities and associations
between minor impairments and subtle structural brain
abnormalities.
OBSTETRICAL BRACHIAL PLEXUS PALSY:
OLD PROBLEM, NEW UNDERSTANDING
Michel R. Magistris
Geneva University Hospital, Geneva, Switzerland
Description of brachial palsy of infants dates back to Hippocrates time. Relation to birth and obstetrics is first considered
in the mid-1700. The term Obstetrical Brachial Plexus Palsy
(OPP) is proposed in 1872. OPP relates to traction on the
brachial plexus during delivery causing brachial palsy. Extension of the palsy may concern roots C5-C6, C5-C7, C8, Th1.
Recovery depends on the 3 possible types of nerve injury. Extremes are represented by neurapraxia (conduction block
caused by compression) that recovers completely in less
than 3 months (most frequent situation), and by neurotmesis
or root avulsion which have no spontaneous potential to recover. Recovery from axonotmesis depends on the proportion of dis rupted ax ons. If reinnervation by col lat eral
i n t r a - m u s c u l a r s p r o u t i n g i s i n s u f fi c i e n t , t e r m i n a l
reinnervation occurs that may lead to incomplete and faulty
reinnervation. This ex plains (i) per sis tent weak ness,
(ii) co-contractions (abnormal syncinesis) restricting intended movements, and (iii) therapeutic limitations. Evaluation of extension, type of nerve fiber damage and subsequent
re gen er a tion re quires care ful clin i cal, ra dio log i cal and
electrodiagnostic investigations. The latter are difficult to apply to in fants but should be con sid ered at later age.
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Electrophysiology has demonstrated the major role in sequel
of OPP of faulty reinnervation causing simple misdirection
(axon that reinnervates wrong muscle) and complex misdirection (axon sprouts that invades 2 or several motor or sensory nerves). Understanding of these phenomena is mandatory to explain the particular condition of a patient and to envisage the appropriate treatment. Surgery started at the beginning of the 19th century and continued until 1930. It was
stopped due to poor results and replaced by conservative
measures which are similarly inefficient. Attempts using
microsurgical techniques are being undertaken but efficient
management remains a challenging question. Selection of
patients most likely to benefit from microsurgical intervention
may derive from scrupulous electrophysiological and non-invasive functional tests.
SPASTICITY IN CHILDREN
Anita Vetra
National Rehabilitation Center, Jurmala, Latvia
Clinical expierence with botulinum toxin A (BtA) in the treatment of spasticity in children started in early 1990s with the
first trials reported by Koman and Graham and coworkers.
Several studies have documented efficacy, safety and sideeffects. BtA injections result in focal, temporary chemodenervation of muscles, which can lead to functional gains. The
expression ‘spastic motor disorder’ is used as an umbrella
term. It refers to complex disorders involving cortical, spinal
and musculosceletal systems. The most common clinical
syndrome is cerebral palsy (CP). Spasticity is only one component of the upper motorneurone syndrome. Whatever the
cause, local administration of BtA may temporarily reduce
muscular hyperactivity. There are several important questions when consider treatment with BtA: suitability of local
therapy, indications for treatment, patient selection, timing of
treatment, interdisciplinary treatment- combination therapy.
There are many published studies reported evidence of benefits from using BtA in pediatric spasticity. BtA improve posture, spasms, function and ease of care, and may reduce the
need for other tretment. In the last few years several randomised control trials of BtA for spastic equinus have been completed. Many experts believe that BtA is helpful for arm
spasticity, but controlled data come only from few small
studies and more trials are needed. The most common deformity in CP is spastic equinus and the most frequent indication for the use of BtA is dynamic equinus, but management is complicated by the fact that not every child has a true
equinus gate. The goals of managing spastic equinus vary
from facilitating independent walking to better tolerance of
orthosis. Evaluation of the outcome is essential, because
treatment with BtA is a relatively new. BtA treatment should
be given as a part of spasticity service and should not be
used in isolation.
IMPROVEMENT OF FUNCTIONAL ABILITIES
OF CHILDREN WITH CEREBRAL PALSY
TREATED WITH BOTULINUM TOXIN
Laima Mikulenaite, Ruta Samaitiene, Liuda Istigeceva
Vilnius University Children Hospital Child Development Center,
Vilnius, Lithuania
Introduction: Botulinum toxin A (Dysport) is being used for
treatment of children with spasticity to reduce impairment and
functional limitations. Previously for many years treatment of
cerebral palsy has centered on the use of physiotherapy and
orthotics and some medications to overcome the problems of
leg spasticity.
Purpose: To evaluate the effect of treatment of children with
spastic cerebral palsy to functional limitations associate with
disability in daily life.
Patients, methods: 54 patients (age range 2–9 years),
treated in Child Development Center, Vilnius, Lithuania with
spastic cerebral palsy were enrolled. The outcome measure
was observational gait analysis, the modified Ashworth Scale,
range of motions, Selective Motor Control Scale, Gross Motor
Function measure (GMFM-66), sitting scale and functional
abilities scale. The indication for injection was the presence of
a dynamic contracture of lower-limb muscles interfering with
positioning or walking. Spastic target muscles were identified
by clinical examination. The effects of injection were monitored by repeated clinical examination.
Results: Improvements have been documented in tone reduction, range of motion, gait pattern, sitting and functional
abilities interfering with daily life of patient.
THE OCCURRENCE OF OBSTRUCTIVE
VERSUS ABSORPTIVE HYDROCEPHALUS
IN NEWBORNS AND INFANTS: RELEVANCE
TO TREATMENT CHOICES
Liana Beni-Adani, Naresh Biani, Liat Ben-Sirah,
Shlomi Constantini
Dana Children’s Hospital, Tel-Aviv Sourasky Medical Center,
Israel
Objective: The classification of hydrocephalus in newborns
and in infants is different from the classification in adulthood.
The objective of this paper is to present the spectrum of obstructive-communicating Hydrocephalus, which is more complex in the pediatric group, and to propose the relevance of
this particular classification to treatment options.
Materials and Methods: The authors categorized infants
with active hydrocephalus at time of presentation into the
following 4 groups along the spectrum of communicating
versus obstructive HCP. Group 1: Patients with a purely absorptive (communicating) HCP. In these patients, 4-ventricular dilatation is usually observed, with occasional extra-axial
fluid accumulation. An extra-cranial CSF diversion (shunt) is
the treatment of choice. Group 2: Patients with an obstructive component together with a persistent absorptive component. In these patients, a technically successful endoscopic procedure will not prevent progression of clinical
symptoms of HCP. An extra-cranial CSF diversion (shunt)
should be the treatment of choice, even though some of
these patients are currently treated by endoscopy. Group 3:
Patients with an obstructive component together with a temporary absorptive component. In these patients, a technically successful ETV should be followed by temporary CSF
drainage. Group 4: Patients with a purely obstructive HCP. In
these patients, an endoscopic procedure (ETV) is the treatment of choice.
Results: The data suggests that obstructive hydrocephalus in
the very young population may be rather a combination of obstructive and absorptive problem. The outcome of the patient
depends mainly on the basic pathology causing the hydrocephalus but also on the treatment that is chosen and its complications. In transient absorptive hydrocephalus, temporary
measures were effective in many cases leading to successful
procedures of ETV and / or posterior-fossa decompression in
selected cases.
Conclusions: Understanding the basic pathology in a given
child may open the window of opportunities for other than
shunt surgery in many hydrocephalic children with major obstructive component.
INTRACRANIAL PRESSURE TARGETED
TREATMENT OF SEVERE PEDIATRIC HEAD
INJURY: LITHUANIAN EXPERIENCE
Dovile Grinkeviciute, Vytautas Ragaisis
Kaunas University of Medicine, Kaunas, Lithuania
Background. Despite preventive measures traumatic brain injury remains the major injury-related mortality and morbidity
factor among children. Outcome is a consequence of the initial impact and secondary injury mecanisms. Intracranial pressure targeted therapy is based on physiological principles for
volume regulation of the intracranial compartment. The essential goal of this therapy is to maintain cerebral perfusion pressure (CPP) above certain level. In Pediatric Intensive Care Unit
(PICU) of Kaunas Medical University Clinic (KMUC) we started
applying the protocol of severe head injury treatment in 2003.
Method. This was a retrospective study of all patients after severe head injury treated over a period of 1999–2006. We compared the outcomes after severe head injury before and after
2003 (1-st and 2-nd groups) on discharge and after 6 months
using Glasgow Outcome Scale (GOS). Head injury was defined as severe if postresuscitation Glasgow Coma Scale
(GCS) £ 8. Poor outcome referred to GOS £ 3, good outcome
referred to GOS > 3.
Results. 137 children after severe head injury were involved
in the study. Intracranial pressure (ICP) was measured in
4 (6,5%) cases in first group and in 36 (48%) in second group.
Decompressive craniectomy was performed in 23 (37,1%)
and 32 (42,1%) cases. Mortality rate was 33,9% in the first and
29,9% in the second group. Totally 95 (69,3%) patients survived, and outcomes were evaluated only for survivals. On
discharge poor outcome was applied to13 (31.7%) and good
outcome to 28 (68.3%) patients in the first group and subsequently 26 (48.1%) and 28 (51%) patients in the second
group. The difference between the groups was not statistically significant (p>0.05). But after 6 months the difference
was statistically significant: 8 (19.5%) and 33 (80.8%) for the
first group and 3 (5.6%) and 51 (94.4%) for the second group
(p<0.05) Conclusions.Treating pediatric patients according
intracranial pressure targeted therapy results in a favourable
long-term outcome.
INFLICTED TRAUMATIC BRAIN INJURY (ITBI)
OR SHAKEN BABY SYNDROME (SBS)
IN ESTONIA: INCIDENCE AND LONG-TERM
OUTCOME
Inga Talvik, Mairi Mannamaa, Tiina Talvik
Children’s Clinic of Tartu University Hospitals, Tartu, Estonia
Inflicted traumatic brain injury (ITBI) or shaken baby syndrome is recognized as a major cause of disability and death
in the paediatric population. The aim of the study was to investigate the incidence rate of ITBI in Estonia and long-term
outcome Patients and methods During the period from January 1st 1997 and December 31st 2003 a total of 26 children
met the criteria of ITBI: 4 children died, 22 survived. All survivors were examined during the years 2005–2006 by the team
of: neurologist, psychologist and an ophthalmologist.
Results: The study group in incidence study included 26 children, 20 (77%) were boys and six (23%) were girls, 4 (15,4%)
of them died Median age at admission to hospital was 3.9 mo,
and the boys were younger than the girls. The overall incidence of ITBI was 28.7 per 100,000 infants. In the prospective
group the incidence was 40.5 per 100,000, and in retrospective group 13.5 per 100,000. The study group for follow up included all 22 children, 18 (82%) boys, 4 (18%) girls. The mean
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age of chil dren at fol low-up was 5,2 years (range
2,3–9,0 years). The follow – up period was 4,67 years (range
2,3–8,6 years) Severe motor problems were in 5/22 children
(22%), 13/22 had light to moderate neurological symptoms.
One child was autistic. Epilepsy was diagnosed in 7/22 cases
(32%). Only 2/22 (9,1%) children were without any developmental problems at the follow-up. 17/22 (77%) had delayed
mental development.
In conclusion. The incidence of ITBI in Estonia was 40.5 per
100,000. Only 2/22 children were without any problems in follow-up and 20/22 had problems with different severity. More
educational programs for prevention of abusive behavioural
of parents.
PEDIATRIC BRAIN TUMORS IN LITHUANIA:
EPIDEMIOLOGICAL OVERVIEW
Rosita Kiudeliene, Giedre Rutkauskiene,
Algimantas Matukevicius, Donata Jurgaityte,
Lingvita Gumbeleviciene, Nerija Vaiciene
Kaunas University Hospital, Kaunas, Lithuania
Background. Brain tumors are the most common solid tumors that occur in children. Up to date there is no published
epidemiological data on pediatric brain tumors in Lithuania.
Aim of the study was to analyze incidence, morphological
type, treatment modalities and mortality rate in children with
brain tumors.
Methods. We retrospectively analyzed medical records of
171 cases of primary brain tumors that within seven years period (2000–2006) were diagnosed and treated at Kaunas
Medical University Hospital which serves as the only hospital
dealing with pediatric neurooncology in Lithuania.
Results: The incidence of brain tumors was 16–32 new cases
per year. The age of patients ranged between 10 months–
17 years (mean 8.68±4.97 years), 93 (54.4%) were boys,
78 (45.6%) were girls. Low grade gliomas constituted 25%,
medulloblastomas/PNET – 23%, high grade gliomas – 20% of
all brain tumors. Radical surgery was performed in 32.9%,
partial in 34.7% of cases, biopsy only were taken in 15.3% of
cases. Surgery was not performed in 17.1% of patients: 5.3%
were given chemotherapy, 11% – radiotherapy only. 23.4% of
children with brain tumors received complex management.
25.7% of patients died within 1–84 months (mean 38.2±25.8
months) period since the moment of diagnosis, 66.7% of patients survived, mainly with low grade gliomas, germ cell tumors and craniopharyngeomas, 25.7% of patients died:
76.9% with brainstem tumor, 60.9% with medulloblastoma/
PNET, 60% with high grade gliomas.
Conclusions. Incidence of brain tumors in Lithuania is
3/100.000; male-female ratio is 1.2:1. Low grade gliomas are
the most common malignancies causing low death rate.
Complex management of brain tumors in Lithuania is still not
sufficient due to recent introduction of chemotherapy.
EPILEPSIES OF CHILDHOOD: DIAGNOSTIC
CHALLENGES
Marina Nikanorova
Danish Epilepsy Center, Dianalund, Denmark
Besides the rather clear-cut guidelines of ILAE Classification
of Epilepsies and Epileptic Syndromes, their diagnosis in
childhood is often a challenge. It might be due to several reasons: some patients cannot be given a recognized syndrome
diagnosis; syndromes overlap or evolve one from another;
syndromes change as new information is obtained; terminol-
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ogy is complex or inappropriate. Some proportion of epileptic
syndromes end up being classified in non-specific categories – “cryptogenic lo cal iza tion-re lated epilepsies” or
“epilepsies without unequivocal generalized or focal features”. It should be taken into account that many epileptic
syndromes are common and well defined, others need further
research and categorization. There are as well syndromes
where genetics and pathophysiology have been dramatically
clarified (BFNC), some new single-gene syndromes described in the recent years and new syndromes reported only
by several investigators (migrating focal seizures of early infancy). Another pitfall of the current classification is terminology which not always is relevant. The dichotomic subdivision
into “localization-related” versus “generalized” epilepsies
might be difficult in some cases, as there are rather many conditions lying between these 2 categories (encephalopathy
with CSWS). Term “focal” should be used in respect of seizures but not syndromes (all patients with localization-related
epilepsies have focal seizures but not all have focus – BECTs).
The other challenge is to determine to what extent we have to
split epilepsies into different syndromes.
FRONTAL LOBE EPILEPSIES IN CHILDHOOD:
SEMEIOLOGY AND CLINICAL SPECTRUM
Walter van Emde Boas
Stichting Epilepsie Instellingen Nederland, Heemstede,
The Netherlands
Frontal lobe epilepsies are defined as localisation related
epilepsies or epilepsy syndromes characterised by simple or
complex partial and/or secondary generalised seizures emanating from the frontal lobe. They constitute a heterogeneous
group of seizure disorders characterised by a variety of seizure types, EEG patterns and aetiologies. Their exact prevalence is unknown but they may constitute up to 1/3 of all
epilepsies in adults and probably as much or even more in
children. Up to seven different types of frontal lobe epilepsy
are recognised in the 1989 ILAE classification of epilepsies
and epileptic syndromes, dependent on their (presumed) area
of seizure onset within specific area’s of the frontal lobes and
on specific ictal signs and symptoms, known to relate to ictal
activity in that specific area. Most are characterised by predominant motor signs, sometimes involving massive and at
times bizarre hypermotor behaviour which may suggest nonepileptic or psychogenic behaviour, but behavioural arrest
(mimicking absence seizures), autonomic signs and symptoms and emotional changes, notably fear, may be an expression of frontal lobe seizures. Due to the intricate neuronal networks connecting the frontal lobe to other brain areas and
within the frontal lobe itself and due to the propensity of very
fast propagation of ictal activity through these networks, the
assessment of these epilepsies is not always easy, especially
since the MRI in many of these patients is negative and the
EEG often is relatively non-contributory, may be misleading or
can actually suggest generalised epilepsy. In children the differential diagnosis is further hampered by the fact that ‘typical’
frontal lobe or temporal lobe semeiology is often lacking during the first decade. Frontal lobe seizures can present with
‘typical’ bilateral spike and wave patterns, at times making differential diagnosis with idiopathic generalised epilepsies extremely difficult. Moreover there is increasing evidence that at
least some of the archetypal idiopathic generalised epilepsies,
including childhood absence epilepsy, express themselves in
seizures which involve relatively limited neuronal circuits, including the frontal cortex and some thalamic nuclei, rather
than the hemispheric cortex as a whole. The clinical spectrum
of frontal lobe epilepsy thus is evolving and may come to include epilepsy syndromes that, at this point, are considered to
be generalised rather than localised.
MALFORMATIONS OF CORTICAL
DEVELOPMENT: IS PROGNOSIS OF
EPILEPSY PREDICTABLE?
Nebojsa Jovic
Clinic of Neurology and Psychiatry for Children and Youth,
Belgrade, Serbia
Malformations of cortical development (MCD) are often recognized as underlying etiology for intractable seizures in children. Early prediction of clinical course and identification of
pharmacoresistance are challenge in their management.
Methods: A Group of 212 children and adolescents aged 0.5
to 17 years with MCD was prospectively studied (3–9.5 years)
for clinical course, prognosis and seizure control. Various
MCD were disclosed by MRI techniques and classified
(Barkovich, Brain Dev 2002; 24: 2–12).
Results: Seizures occurred in 167 (78.8%) MCD patients with
mean seizure onset of 3.6 years. Mental retardation was found
in 61.3% of children. Focal neurologial deficits were noted in
91 patients. Generalized epileptic syndromes were diagnosed in 64, partial epilepsies in 103 patients. Epileptic status
occurred in 17.9% epileptics. Seizures refractory to AEDs occurred in 43.1% patients (Group A). Complete long-term seizure control was achieved in 18.6%, till >75% seizure reduction was noted in 38.3% (Group B). New AEDs were effective
in 56 patients. MCD with multilobar involvement, hemimegalencephaly, lyssencephaly 1, subcortical heterotopias and
perysylvian polymicrogyria were more often associated with
intractable epilepsy in comparison with partial schizencephaly, focal CD, TSC and unilateral pahygyria. Seizure onset
was significantly earlier in a Group A (mean 2.3 yrs) when
compared with Group B (7.2 yrs). Infantile spasms occurred
in 13.8% of children with later pharmacorestance vs. 4.8% of
children from Group B. Favourable initial therapeutic response was significantly higher in a Group B (58/95) vs.
Group A (16/72). Pharmacoresistant seizures were most often
associated with mental retardation, sensory impairment and
undeveloped speech before epilepsy and multifocal/bilateral
EEG abnormalities when compared with well-controlled patients.
Conclusions: Early seizures onset, occurrence of infantile
spasms, poor response to initial treatment, multilobar/bilateral MCD and early development of severe clinical condition
were identified as main predictors of seizure intractability.
SHOULD WE TREAT EPILEPSY?
Jurgis Strautmanis
Childrens Clinical University Hospital, Riga, Latvia
Background. There is a popular opinion among Latvian parents of epilepsy patients that seizures are rather beneficial
than harmful, but anticonvulsants are noxious and should be
avoided. This theory comes from rehabilitation center of Philadelphia’s “Institutes for the achievement of human potential”
(IAHP).
Methods. Search of the sources of this theory and counterarguments in the literature.
Results. The theory that seizures are beneficial originates in
publication of Temple Fay (1942). He raised hypothesis that
seizures have been developed during evolution as reflex reaction to hypoxia with aim to help early amphibians get back in
water if their abilities to get oxygen in land would appear insufficient. It’s based on observations that sei zures occur
throughout animal world, can be induced in any brain and
ictal movements are typical to amphibians (simple flexor –
extensor type, never complex). Therefore he considered sei-
zures as reflex reaction to hypoxia. G. Doman, R. Pelligra
added to the theory novel data about relationship of neuronal
activity and cerebral blood flow regulation, role of ATP dependent ion pumps in the ictogenesis and the fact that there is
hyperperfusion, hypermetabolism during seizures and hypoperfusion, hypometabolism in interictal period in epileptogenic region. If we look at such approach critically we can find
little data in the literature that seizures could be beneficial. On
the contrary there are lot of reports that seizures can induce
brain lesion, predicts higher trauma and mortality risk, is associated with social and psychiatric problems and this is true
even in idiopathic epilepsy despite difficulties to separate effects of underlying diseases and seizures.
Conclusions. I suppose presented theory of ictogenesis is interesting and demands further discussion in scientific literature. Unfortunately it is used in malicious manner in IAHP.
There is little data that seizures could be beneficial but lot of
data that seizures could be harmful.
CURRENT VIEWS ON PEDIATRIC
PSYCHOGENIC NON-EPILEPTIC SEIZURES
Sigita Plioplys
Children’s Memorial Hospital, Chicago, USA
Objective: To review current research data on epidemiological, clinical and developmental characteristics of pediatric
psychogenic non-epileptic seizures (PNES).
Background: Children with PNES are often undiagnosed and
untreated, because of the difficulties in timely diagnosis and
lack of standardized treatment guidelines. While searching for
the “correct” diagnosis, these patients are frequently exposed
to unnecessary medical procedures and treatments, which
may cause iatrogenic side effects, significant economical burden to the families and high utilization of health care services.
Methods: Data on prevalence, risk factors, clinical and developmental characteristics, comorbid psychopathology and
long term outcome of pediatric PNES will be discussed.
Psychodynamic, Behavioral, and Family Systems theories of
PNES development will be reviewed. Strategies for the outpatient multidisciplinary treatment will be presented.
Results: The prevalence of pediatric PNES as reported from
the Epilepsy Monitoring Units vary from 11% to 30%. It is similar in pre-pubertal boys and girls, but becomes significantly
higher in adolescent girls. The median delay in PNES diagnosis is about 3.5 years. At the time of the initial PNES diagnosis,
50–95% of children are taking antiepileptic drugs. A history of
sexual abuse is commonly reported in older teenage girls and
physical abuse is more frequently identified in younger children. Comorbid epilepsy is more frequently present in children younger that 5 years (48%) as compared to children
5–12 years old (25%), and adolescents (19%). The most common psychiatric diagnoses identified in youth with PNES are
Conversion Disorder (77%), Attention Deficit Hyperactivity
Disorder (64%), and Major Depressive Disorder (18%). Predictors of positive PNES outcome are younger age at onset,
female gender, and multiple “seizure” types.
Conclusions: Standardized and population based research
studies are needed to generate evidence based clinical data
for standardized PNES diagnostic and treatment practice
guidelines.
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DIAGNOSIS AND FEEDBACK OF PEDIATRIC
NON-EPILEPTIC SEIZURES (PNES) DO’S
AND DON’TS
Rochelle Caplan
UCLA, Los Angeles, USA
Objectives: This presentation will describe diagnostic assessment techniques that identify psychopathology in children and adolescents with PNES. It will also present clinical
guidelines for the effective presentation of a PNES diagnosis
to these children and their families. The importance of making
a timely PNES diagnosis will be discussed.
Methods: A clinical discussion will walk participants through
the diagnostic process from when children with PNES are first
seen by the neurologist until completion of the assessment of
PNES. Brief presentation of clinical cases, in which diagnosis
and feedback were successfully and unsuccessfully managed, will support recommendations on “do’s and don’ts”
when diagnosing children with PNES.
Results: Youth with PNES are often undiagnosed and the underlying emotional causes of their condition go untreated.
Therefore, PNES has marked morbidity with repeated ER and
physician visits, school absenteeism, missed days of work for
parents, and unnecessary use of high doses of multiple
antiepileptic drugs. This presentation will alert pediatric neurologists to the importance of including PNES in their differential diagnosis of all youth with epilepsy. It will also provide
techniques to help pediatric neurologists obtain valid clinical
information from these patients and their families, as well as
develop new skills for timely diagnosis of PNES.
Conclusion: With this information, pediatric neurologists will
feel better equipped to diagnose PNES and provide feedback
to these children and their families.
ADHD: ALTERATIONS OF DOPAMINERGIC
SYSTEM
Aurelija Jucaite
Karolinska Institute, Stockholm, Sweden
The hypothesis that altered dopamine transmission underlies
hyperactive-inattentive behaviour in children with AttentionDeficit/Hyperactivity Disorder (ADHD) is based on genetic
studies, efficacy of psychostimulants, animal models of hyperactivity. Molecular imaging studies, investigating central
dopamine system in humans in vivo, have focused so far on
binding of dopamine markers in the basal ganglia, midbrain.
We investigated dopamine D1 receptor (D1R) density in
ADHD children using positron emission tomography and
radioligand [11C]SCH23390. Thirteen children with ADHD
and twelve comparison children, 9 to 17 years of age participated in the study. Attention and motor behaviour were investigated with a continuous performance task (CPT) and motion
measurements, reaction time, visuo-spatial working memory,
Stroop test were included in the neurocognitive test battery.
The [11C]SCH23390 binding to D1R was decreasing with age
in both groups, with highly significant decline in ortical regions in the group of children with ADHD, suggesting intensive and widespread biochemical changes in cortical networks during adolescence.
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PREVALENCE AND CURRENT TREATMENT
OF ATTENTION DEFICIT – HYPERACTIVITY
DISORDER (ADHD) IN LITHUANIA PRIMARY
SCHOOL PUPILS
Darius Leskauskas
The goal of a study was to establish the prevalence of attention deficit – hyperactivity disorder (ADHD) in the primary
school children in Lithuania and to evaluate what treatment
is currently provided for them. Parents and teachers of all pupils of 12 randomly selected primary schools (N=2942)
completed the scales on the presents of ADHD symptoms in
their children. ADHD was diagnosed if at least 6 symptoms of
hyperactivity and/or 6 symptoms of inattention were present
for at least 6 months according reports of parents and the result of CAP scale completed by teachers exceeded the
norms for age and gender. Results of the both scales were
ob tained for 2298 pu pils – 1096 girls and 1202 boys
6–12 years of age. ADHD was diagnosed for 5.2% of primary
school pupils. It was 1.9 times more frequent among boys
than among girls. There was no significant difference between the four grades in the incidence of ADHD in general
but there was a relationship between the age of the pupils
and the prevalent type of ADHD. Statistically significant relationship was established between ADHD and worse achievements in learning, problems in communications with peers
and parents, oppositional defiant behavior. 51% percent of
the parents of ADHD diagnosed children have had applied to
the mental health care professionals, most of them evaluated the help they received as just partly useful (57.1%) or
not useful at all (24.5%). Most of those children had received
one consultation of a specialist (17.3%), were offered just
psy cho ther apy / be hav ioral ther apy (16.3%) or just
pharmacotherapy (8.1%). Only 5.1% of them had received
complex treatment.
COGNITIVE DYSFUNCTION OF CHILDREN
WITH SYMTOMATIC AND NEWLY
DIAGNOSED EPILEPSY
Anneli Kolk
Children’s Clinic of Tartu University Hospital, Tartu, Estonia
Background: The aim of this study was to specify the neuropsychological outcome in case of children with the unilateral
nonprogressive brain lesion.
Method: In order to assess these specific functions, we used
a comprehensive model of neonatal stroke with focal epilepsy and newly diagnosed focal epilepsy. The neuropsychological examination was performed using the NEPSY test battery on 44 children aged from 4 to 12 years. The children
were divided into three groups: 18 children suffering from
neonatal stroke and focal epilepsy, 12 children with newly diagnosed focal epilepsy prior to antiepileptic treatment, and
14 healthy controls matched by sex, age, and socio-economic status.
Results: Children with neonatal stroke and epilepsy had
more severe cognitive deficit, especially in language, visuoperceptual, memory and learning tasks than children with
newly diagnosed partial epilepsy. However, the profile of the
cognitive weakness appears to be diffuse and quite similar
with both groups. The children with newly diagnosed epilepsy
did not demonstrate a clear effect of lateralization, according
to the side of epileptic EEG discharges. Especially interesting
was the fact that the newly diagnosed epilepsy group showed
impairment not only in attention, visuoperceptual and short-
term memory skills, but also in auditory perception, lexical
function, and the comprehension of speech.
Conclusions: It is highly recommended that the children with
epilepsy would undergo a neuropsychological examination in
order to assess their later cognitive development.
22 June, Friday
SEIZURE SEMIOLOGY AND SYNDROMIC
APPROAH IN CHILDREN UNDER 3 YEARS
Perrine Plouin
Hopital Necker Enfants Malades, Paris, France
In the classification of epilepsy syndromes accepted in 1989,
5 syndromes were recognized in infancy, none of them with
focal seizures. In 2001 eleven syndromes were proposed,
and the 6 new ones were concerned with focal seizures. First
step is to classify seizures, second to recognize syndromes
with clinical, EEG, neuroradiological and genetic data. This
improvement in our knowledge on the semiology of seizures
in infancy is due to development of longterm EEG video monitoring. Simultaneous polygraphy helps in classification of
myoclonic, atonic or tonic components of seizures as well as
spasms. In infancy epileptic spasms are the most frequent
seizure type; around 1980 were reported focal seizures associated with spasms and asymmetrical spasms; both situations are linked with symptomatic cases although symmetrical spasms are associated with idiopathic or cryptogenic
West syndrome. Myoclonic seizures are present in several epilepsy syndromes among neonates and infants. They may be
massive, segmentary or erratic. Some of these syndromes
are severe such as Early Myoclonic Encephalopathy occurring in neonates, sometimes associated with metabolic diseases. In Benign Myoclonic Epilepsy of Infancy (reflex or not)
which belong to IGE, myoclonic seizures are massive as well
as in Dravet syndrome or Doose syndrome both cryptogenic.
Myoclonus status epilepticus is characterized by erratic myoclonusas in Angelman syndrome but also in progressive neurological diseases as ceroidolipofushinosis. Focal seizures
have been precisely analyzed according to the localization
and one can easily recognize a frontal, from a central, temporal or occipital seizure in this population where subjective
signs are not reported. Benign familial and non familial syndromes with focal seizures should be differenciate from
symptomatic focal epilepsies and from epileptic encephalopathies comprising only focal seizures such as MPSI. Recognizing the type of seizures in syndromes in infants is an important challenge considering the etiology, the prognosis and the
treatment.
PANAYIOTOPOULOS SYNDROME VS
GASTAUT-TYPE OCCIPITAL EPILEPSY
Anthanasios Covanis
The Childrens Hospital “Agia Sophia”, Athens, Greece
The ILAEs 2001 Diagnostic Scheme recognised two childhood occipital epilepsies, including early onset type benign
childhood occipital epilepsy and late onset childhood occipital epilepsy. However, during the last decade independent
studies have suggested, that these conditions are likely to be
separate disorders, rather than variants of a single disorder
and that whilst seizures in Gastaut type occipital epilepsy are
indeed likely to originate in the occipital lobes, this is unlikely
to be the case in Panayiotopoulos syndrome(PS). An expert
consensus defines PS ‘as a benign age-related focal seizure
disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an EEG that shows shifting and/or
multiple foci, often with occipital predominance.’ PS probably
affects 13% of children aged 3–6 years who have had one or
more afebrile seizures and Gastaut-Type 2–7% of the benign
childhood focal seizures. Panayiotopoulos syndrome occurs
in children who are otherwise normal and is not associated
with the development of significant neurodevelopmental
problems. Autonomic epileptic seizures and autonomic status epilepticus are the cardinal manifestations. Characteristically the child, who was initially fully conscious, becomes
confused, unresponsive and the eyes turn to one side or gaze
widely open. Half of the seizures in PS last for more than 30
minutes, thus constituting autonomic status epilepticus,
which is the commoner non-convulsive status epilepticus in
normal children. Two thirds of seizures occur during sleep,
particularly the early part of sleep. Gastaut-type idiopathic
childhood occipital epilepsy has entirely different clinical
manifestations, despite common interictal EEG features when
occipital paroxysms occur in PS. Visual hallucinations, elementary or complex, are the prominent seizure type in
Gastaut type and the ictal EEG, when performed is also markedly different. Visual seizures are predominantly diurnal and
occur at any time of the day Prognosis in PS is remarkably benign in terms of seizure frequency and evolution while in
Gastaut-Type the prognosis is unclear.
PSYCHIATRIC AND COGNITIVE
COMORBIDITIES IN PEDIATRIC EPILEPSY:
CAUSE OR EFFECT?
Rochelle Caplan
UCLA, Los Angeles, USA
Numerous studies demonstrate high rates of a wide range of
psychopathology and subtle to marked cognitive difficulties
in children and adolescents with both new onset and chronic
epilepsy. Despite consistent evidence for a relationship between seizures variables and the cognitive comorbidities of
pediatric epilepsy, findings are inconsistent regarding their relationship with psychopathology. This presentation will review
studies conducted during the past decade demonstrating the
comborbidity of behavioral, cognitive, and linguistic deficits in
children with epilepsy. It will discuss the types of epilepsy
and/or epilepsy syndromes that are related to specific types
of psychopathology and patterns of cognitive and linguistic
deficits in these children. Using a developmental model, it will
also focus on studies that have examined the role played by
seizure related factors and underlying neuropathology in
these comorbidities. After a brief discussion of methodological issues in the reviewed studies, it will present a model of
epilepsy as a developmental neuropsychiatric illness that
presents with different types of seizures, varying seizure control, as well as a wide range of behavioral disturbances and
cognitive deficits.
PHARMACOLOGICAL TREATMENT OF
ATTENTION DEFICIT HYPERACTIVITY
DISORDER IN CHILDREN WITH EPILEPSY
Sigita Plioplys
Children’s Memorial Hospital, Chicago, USA
Objective: To review current pharmacological treatment strategies for children with Attention Deficit Hyperactivity Disorder
(ADHD) and epilepsy. Background: ADHD negatively affects a
child’s academic achievement, behavioral and emotional
S23
functioning, and social interactions. Medications, in addition
to educational, behavioral and family interventions, are effective ADHD treatment options.
CEREBELLAR ATROPHIES
Methods: Research data and clinical information on pharmacological treatment strategies of ADHD in children with epilepsy is presented.
University Children’s Hospital, Zurich, Switzerland
Results: ADHD symptoms are reported in 37% of elementary
school age children and 25% of adolescents with epilepsy. It
is important to establish if there is a temporal association between the development of ADHD symptoms and the course
and treatment of epilepsy. It will allow clinician to determine if
inattentiveness, hyperactivity and impulsivity are independent
developmental symptoms, i.e. ADHD, or represent epilepsyassociated phenomena, such as new onset absence seizures, unrecognized nonconvulsive seizures, or side effects
of antiepileptic medications. Further treatment strategies
should be determined by this association. Controlled data are
available only on the efficacy and safety of methylphenidate
(MPH) for the use in children with epilepsy. No published data
on the seizure risk are available for Atomoxetine, Guanfacine
or Clonidine. Due to the risk of causing or worsening seizures
at moderate to high doses, Bupropion is not recommended
for children with epilepsy.
Conclusions: MPH is effective and safe medication for the
treatment of ADHD in children with well controlled epilepsy.
CURRENT ACHIEVEMENTS IN THE
GENETICS OF THE EPILEPSIES
Ulrich Stephani, Hiltrud Muhle, Sarah von Spiczak
Pediatric University Hospital, Kiel, Germany
An imbalance of excitatory and inhibitory mechanisms in the
brain is postulated to be the global pathogenic mechanism in
epilepsies. Since the electrical excitability of membranes
mainly is performed by ions and neurotransmitters it was not
surprising that mutations of ion channels and neurotransmitter receptors recently have been discovered to be associated
with monogenic idiopathic generalised and focal epilepsies
(IGE, IFE): Mutations of channels for calcium ions with childhood absence epilepsies, for potassium ions with benign
neonatal familial convulsions, for sodium ions in generalised
epilepsies with febrile seizures plus, severe myoclonic epilepsy of infancy and others, for chloride ions with different
forms of idiopathic generalised epilepsies, for acetylcholine
receptor subunits with a certain fontal epilepsy, for GABA-receptors with different forms of IGE. Only the mutated genes
for the protein myoclonin1 in juvenile myoclonic epilepsy and
for the leucine rich glioma inactivated protein 1 in lateral temporal epilepsy do not belong to this group. Despite the success of discovering these pathogenic mechanisms in the majority of epilepsies the respective mutations or even polymorphisms could not be found yet. In this context it is noteworthy that common complex diseases such as epilepsy do
not follow classic Mendelian inheritance patterns and it is unlikely that epilepsy is caused by single gene defects. One of
several different hypotheses suggests that the disease-predisposing alleles occur frequently in the population and each
of them contributes little to disease susceptibility (known as
the common variant, common disease – CVCD – hypothesis
acc. to Collins et al. 1997). In order to advance the understanding of pathogenetic mechanisms in epilepsy it seems to
be necessary to further endophenotype the epileptic syndromes e.g. by EEG marker being associated with them. Ongoing research on the photoparoxysmal response, its clinical
association with the epilepsies and the molecular genetic
analyses will be shown.
S24
Eugen Boltshauser
The term Cerebellar Atrophy (CA) implies neuroimaging evidence of secondary loss of cerebellar tissue. CA may be seen
as a residual change resulting from a single “hit” (e.g. an intoxication), or it may represent an ongoing process in the context of a progressive disorder. CA may be global, or more
localised, involving either mainly the vermis, or affecting predominantly the hemispheres. CA can be accompanied by
brainstem / pontine atrophy. Depending from the underlying
disease supratentorial atrophy can be present. Differentiation
between hypoplasia, prenatal onset atrophy and even postnatal onset atrophy is not always possible on a single neuroimaging study. It is well documented that congenital non-progressive ataxias may appear on MRI like CA, with dilated / enlarged forth ventricle and enlarged interfoliar spaces. To complicate the situation further, CA can be superimposed on cerebellar hypoplasia, as seen in some patients with congential
disorders of glycosylation. It is important to recognise that not
all progressive ataxias have CA on neuroimaging (e.g.
Friedreich ataxia, Refum disease), while CA may be demonstrated in patients in whom progressive cerebellar dysfunction cannot be demonstrated in view of their neurological
imparment (e.g. PEHO syndrome). In the pediatric age acquired CA are less prevalent than genetic CA, but can occur in
a large spectrum of conditions, including post-inflammatory,
post-traumatic, post-asphyctic, toxic, paraneoplastic, nutritional (vi tamin B12 de fi ciency), and fol low ing ex treme
prematurity. CA is a feature of a large group of inherited progressive disorders (isolated, or with cerebral atrophy, or with
white matter involvement). The MRI appeareance of “genetic”
CA is mostly nonspecific. Exceptions are the cerebellar cortex
hyperintensities seen in infantile neuroaxonal dystrophy and
Marinesco-Sjögren syndrome. The diagnostic approach
should be based on the individual constellation based on history, age, clinical features, and metabolic screening tests.
GENETICS OF MENTAL RETARDATION:
PRESENT AND FUTURE
Lina Basel-Vanagaite
Schneider Children’s Medical Center of Israel, Petah Tikva,
Israel
Mental retardation (MR) is a highly heterogeneous condition
with a prevalence of 1–3% in the general population. The
psychosocial burden on families with mentally handicapped
children is extensive. Genetic etiologies are found in approximately two thirds of mental retardation cases. MR affects
30–50% more males than females, and X-linked MR is thought
to account for this sex bias. It can be subdivided into syndromic forms, which are characterized by MR and either malformations, dysmorphic features, or neurological abnormalities, and nonsyndromic forms, which are characterized by MR
without any additional manifestations. Etiological diagnosis
and genetic counseling for MR is one of the most difficult challenges faced today by clinical geneticists. Only a few recurrent genetic defects exist, such as Down syndrome and Fragile X syndrome. For nonsyndromic MR, more than 80 loci are
described and 28 genes have currently been identified. For
syndromic MR, more than 290 genes have been identified.
Abnormalities in DNA copy number are frequently found in
patients with multiple congenital anomalies/MR syndromes
and can be detected by array-based comparative genomic
hybridization (array-CGH). We present an overview of recent
achievements in the elucidation of the genetic defects causing MR. A comprehensive strategy to systematically identify
the causative genetic defects in patients with MR using a combination of routinely used and recently developed laboratory
tests is described. Some examples of prevention of MR in
specific populations are given.
FUTURE TREATMENTS OF
NEUROMUSCULAR DISORDERS
Thomas Sejersen
Karolinska Institute, Stockholm, Sweden
“What new promising treatments are around the corner”, we
are often asked and ask ourselves.
This question may be viewed from different perspectives. We
will look at them from two of these: a time aspect and a
pathophysiological aspect.
Looking at future treatments from a time perspective there are
three major levels, diffusely denoted present time, near future,
and distant future. Although dealing with the future we will
have to start at the present time. Do we now treat according to
the best available knowledge? How do we know if we do so?
The task to establish and follow an already present golden
standard of treatment is here a first important step. In the near
future we have treatments in or close to later phase clinical trials that are likely to result in additions in the therapeutic arsenal. In a more distant future we foresee novel therapies that
are now at a state of basic research or early clinical trials, and
also not yet defined strategies. Among these are means of directly compensating for the genetic alteration that often underlie neuromuscular disorders.
If we instead look at future treatment options from a pathophysiological aspect, we can see areas of treatment and care
arising that are directed at 1) counteracting the primary
pathogenetic mech a nism un der ly ing the dis or der (e.g.
corticosteroid treatment of DMD, medical attempts to increase SMN2 levels in SMA, and gene therapies), or 2) symptomatic for an affected tissue or organ (e.g. ACE-inhibitor
treatment of approaching cardiac insufficiency, scoliosis surgery, ventilatory support etc).
Yet another level we need see delevoped in the future is a
global habilitation/rehabilitation view for measures that are supportive for well-being and life in society, including measures to
increase independence, ease communication, and participation in society, e.g. in the educational system and at work.
No matter what perspecitve we look at future measures, a key
feature is that large efforts are needed at a local plane, but
more importantly, at an overall multinational level. Therefore,
the best measure to allow for this improvement in future therapies at various levels is the creation of large international networks, e.g. TREAT-NMD, to implement the best current knowledge, to increase awareness in society, and to collaborate for
finding novel theraputic strategies, and to allow for continuously ongoing, good clinical trials to improve present therapies and test new ones.
INHERITED METABOLIC CAUSES OF
NEUROLOGIC DISEASES IN NEONATES AND
INFANTS
Leo Spaapen
Academic Hospital Maastricht, Maastricht, The Netherlands
Neurologic disturbances are major characteristics of many
well-known inherited metabolic diseases (IMD). IMD are
caused by mutations in genes resulting in a structurally altered inactive protein, often an enzyme. The enzyme defect
may cause a block in a metabolic pathway, resulting in accumulation of a metabolic substrate which may be neurotoxic
and/or deprivation of an indispensable product. In the neonatal period neurometabolic disorders may present with seizures, floppiness, impaired consciousness, feeding problems, hypothermia, facial dysmorphisms, micro- or macrocephaly or hydrops fetalis. In the early infantile life failure to
thrive, poor head control, recurrent infections, intractable
convulsions, hyperventilation (central hyperpnoea), skeletal
deformities and eye problems (impairment of vision, corneal
clouding, nystagmus etc.) are additional symptoms. Often
there is a combination of symptoms. The nervous system is
more often involved in IMD than any other organ because the
immature brain in the newborn is most susceptible to biochemical derangement. There are about 100 IMD which may
come to clinical expression in the first weeks of life. This group
of metabolic diseases comprise genetic defects in the metabolism of amino acids, neurotransmitters, fatty acids, lipids and
cho les terol, car bo hy drates, pur ines and py rimi dines,
creatine, and glycoproteins as well as mitochondrial respiratory chain disorders, urea cycle defects, lysosomal storage
diseases and peroxisomal diseases. Scarcely 20 are amenable to treatment. The presentation of metabolic diseases is often characterized by life-threatening decompensation demanding prompt and resolute action. First of all adequate
samples must be obtained for basic laboratory investigations:
blood gases and plasma electrolytes, plasma glucose, lactate
and ammonia, and urinary keton bodies to cover differential
diagnostics. Results of those analyses should be available
within 30 minutes, at the latest. In addition adequate samples
should be taken for spe cial met a bolic in ves ti ga tion:
acylcarnitines (dried blood spots), plasma amino acids, urinary organic acids. Results of special metabolic investigations, relevant to the diagnosis of potentially treatable metabolic disorders should be available at the lat est within
48 hours [1]. The aim of this presentation is to emphasize the
importance of inherited metabolic disorders to become included in the differential diagnostics of neurologic diseases.
ORPHAN DRUGS AND RARE DISEASES:
PROBLEMS AND OPPORTUNITIES
Arrigo Schieppati
Mario Negri Institute for Pharmacologica Research, Ranica, Italy
There are 4 to 5 thousand different diseases that affect a very
limited proportion of patients. Most of these diseases lack an
appropriate treatment, for several reasons. One reason is the
limited knowledge of pathogenesis and pathophysiology,
which is in turn related to the lack of animal models and to the
difficulty of collecting enough patients to obtain a significant
number of observations. However, there is another important
aspect to be considered: rare diseases occur so infrequently
that most of the times there is no reasonable expectation that
the cost of developing and making available a drug for the
treatment of such diseases will be covered by sales. Patients
with rare diseases have been named the “Health Orphans”
and the treatments that could cure them ‘Orphan Drug’. Orphan Drug legislations in US offer incentives consistingn in
market exclusivity for orphan designated products with Food
and Drug Administration approval, and research tax credits
for rare disease clinical studies. Before 1983 only 7 drugs
were available to treat rare disease. Since then, more than
1600 drugs and biologics have been designated as orphan
products and more than 200 of these have received FDA marketing approval. The European Union after lengthy proceedings and delays has finally approved a Regulation for Orphan
Medicinal Products. The European regulation is applicable to
disorders with a prevalence of no more than 5 per 10,000, or
for a life-threatening or seriously debilitating communicable
disease even when the prevalence is higher. The European
regulation, although has been approved after a long awaiting,
S25
has nonetheless raised few criticisms from the industry and
from the academia. These criticisms notwithstanding, patients with rare diseases have now much more opportunity for
treatment than they before in America and in Europe, although many problems are still to be solved.
ACCESSING INFORMATION ON ORPHAN
DRUGS: THE EUORPHAN SERVICE
Paola Baiardi 1, Manuel Posada de La Paz 2,
Lars Savendahl 3, Domenica Taruscio 4, Renza Barbon
Galluppi 5, Enrico Morten 6, Donatas Stakisaitis 7,
Adriana Ceci 8
1
Consorzio per Valutazioni Biologiche e Farmacologiche, Pavia, Italy;
Foundation for Cooperation and International Health Carlos III, Spain;
3
Karolinska Institutet, Stockholm, Sweden;
4
Istituto Superiore di Sanita, Italy;
5
UNIAMO, Federazione Italiana Malattie Rare, Italy;
6
Softeco Sismat S.p.A., Italy;
7
Vilnius University, Vilnius, Lithuania;
8
Consorzio per Valutazioni Biologiche e Farmacologiche, Italy
2
Approximately 30 million people currently suffer from rare diseases in Eu rope. De spite the reg u la tory in ter ven tions
(141/2000/EC Regulation) to propel the development of
drugs for the care of rare disorders (orphan drugs), few treatments are still available and patients find many difficulties in
accessing quality healthcare for their disorders. Moreover,
whenever available, information on orphan drugs is very limited, fragmentary and difficult to retrieve because of the lack of
a unique data source. EuOrphan (www.euorphan.com) is a
service developed under the auspices of the EU eTEN
Programme aimed at distributing a complete set of information on orphan drugs commercialised or still under development (designated drugs) worldwide. The service addresses
all the stakeholders in the field: patients and patient associations, physicians, researchers, public health institutions and
pharmaceutical companies. It provides four main services:
• the access to a centralized database containing standardised information on about 1500 designated and marketed orphan medicines. Availability throughout Europe, prices and
usage in paediatrics are the added value information included
in the database;
• statistics on the orphan market at a worldwide level supporting public health authorities in planning a rationale usage of
resources and public health interventions;
• a consultancy service managed by a team of qualified experts
supporting pharmaceutical companies in developing new orphan drugs and complying with regulatory requirements;
• a forum constituting a useful means to facilitate contacts
among the different actors, to organize pressure groups and
to stimulate new studies. Finally, a news section is updated
monthly and newsletters are provided upon registration quarterly. EuOrphan full service has been running since June
2006. It is currently funded by public and private supporters
that consider access to high quality information a central
means to provide social and ethical benefits to disadvantaged
minorities of patients.
ORPHAN PATIENTS AND ORPHAN
MEDICINES: SITUATION IN LITHUANIA
Indre Spokiene
Mykolas Romeris University, Biolaw Department, Vilnius,
Lithuania
The EU orphan legislation entered into force in April 2000. Orphan medicinal product legislation was timely to address the
S26
unmet medical needs of patients suffering from rare diseases
within Community, as they deserve access to the same quality of treatments as other patients. This legislation is part of a
broader Community policy to identify rare diseases as a priority area for action in the field of public health. Member
States must adopt specific measures to increase knowledge
on rare diseases and to improve their detection, diagnosis
and treatment. Lithuanian measures concerning rare diseases and conditions and national incentives for orphan medicinal products are insufficient. Firstly, the definition of ‘rare
disease or condition’ laid down in the Decree of the Health
Ministry State Patient Fund Directorate do not meet the EU
standards. Secondly, there are no statistical data on the number of patients suffering from rare diseases or conditions in
Lithuania. The other problems: there are no specific patients’
organizations for patients suffering from rare diseases and
their families. There is a lack of information on rare diseases
not only for patients, but also for the health care professionals. These problems are in insignificant part and temporarily
solved through patients’ participation in clinical trials. The
Lithuanian Government promotes the use of orphan medicinal products for specific patients by reimbursement from the
funds earmarked for that purpose in the budget of the compulsory health insurance fund (which is only 5 mll. LTL in
2007). Individuals are compensated for medicinal products
for rare diseases and conditions on presenting specialist
doctors’ reports, following a decision by the Committee approved by the Health Ministry State Patient Fund responsible
for taking decisions regarding treatment of rare diseases and
conditions.
PUBLISHING IN BIOMEDICAL JOURNALS:
HINTS FOR SUCCESS FROM A JOURNAL
EDITOR
Roger Brumback
Creighton University School of Medicine, Omaha, USA
In medical schools, colleges, and universities throughout the
world, academic rank, tenure, and recognition are based in
large part on scholarly achievement, including publication of
case materials and of scientific investigations. Arguments
abound whether quality or quantity of publications is more important (wags have even suggested that “promotion committees can count but not read”). Nonetheless, it is necessary to
understand the process of journal article preparation and
submission to survive the gauntlet of editorial evaluation and
peer review in order to achieve publication. The initial step
should be the appropriate formulation of the question to be
answered in the article. It is imperative to demonstrate the importance of this question in order to justify the addition of this
article to the world’s published biomedical literature. Preparation of the manuscript of the article must follow not only all the
detailed instructions for authors, but also representative published examples from the particular journal to which the
manuscript will be submitted. The first part of the article to be
written is the case summaries and/or methods section which
needs to be comprehensive. The results and discussion sections should then follow, showing what is unique in this article
and stressing why it is worthy of publication. Figures and tables should be prepared in a manner to stand alone, providing sufficient information that reading the article text is not required for understanding. Computer-generated figures must
have sufficient detail and be of high enough quality to be
clearly visible when printed in the journal. The completed
manuscript for submission should be as close to perfect as
possible, since the submitted material will constitute the journal editor’s first impression of the quality of the author’s scholarship.
23 June, Saturday
EPILEPSY, MAGNESIUM, GENDER
DIFFERENCES
Jurgita Grikiniene
Vilnius University Children’s Hospital, Vilnius, Lithuania
Background: Recent research has found gender differences
in prevalence and clinical manifestation of epilepsy in humans, in susceptibility to proconvulsants in animals, genderrelated differences of Mg, Na+ and Cl– metabolism, adverse
drug effects that depend on the gender of patients, but certain
mechanisms of these effects are not clear. Magnesium (Mg) is
involved in the mechanisms of neuronal susceptibility to excitability, including epilepsy. Mg2+ inhibits the facilitating effect
of calcium on synaptic transmission and also exerts a voltage- de pend ent block age of the N-methyl-D-aspartate
(NMDA) receptor. It is well known that low Mg2+ levels induce
spontaneous epileptiform activity in several cortical structures. Several studies suggest that the body electrolytes and
some trace elements may be causally involved in some forms
of epilepsy and also to increase the recurrence of seizures.
Mg urinary excretion in epileptic patients have not been investigated earlier. The aim of the study: to define peculiarities of
urinary Mg excretion in epileptic adolescents without treatment (newly diagnosed epilepsy) and to elucidate gender-related differences.
Methods: We studied 52 untreated epileptic children (newly
diagnosed epilepsy): 23 boys and 29 girls, and 95 control
children (47 boys and 48 girls) aged 11–17 years. Epileptic
children were treated in Department of Children Neurology,
Vilnius University Children’s Hospital. The clinical diagnosis
was made according to the criteria of the International League
against Epilepsy (1981). All studied children did not suffer
from another acute or chronic disease. 24-h urine was collected. Urinary Mg levels were measured with a spectrophotometer. Urinary pH and creatinine levels were measured
also.
Results: 24-h urinary Mg excretion of epileptic girls was significantly higher compared with control girls. These differences were not found in untreated epileptic boys.
Conclusion: Our data showed altered urinary Mg excretion in
epileptic adolescent girls.
FEBRILE SEIZURES AND EPILEPSY:
A RETROSPECTIVE STUDY
Ljerka Cvitanovic Sojat 1, Masa Malenica 1,
Romana Gjergja 1, Zlatko Sabol 2, Tina Sojat 1
1
2
UH Sestre milosrdnice, Zagreb, Croatia;
Pediatric Outpatient Clinic ‘Dr. Sabol’, Croatia
Background: Febrile seizures (FS) precede an onset of various forms of epilepsies in 10–15% of children. Risk for epilepsy in children with FS is 3% by the age of 7 years. Complex
FS are associated with a younger age at onset of epilepsy.
One-third of patients with temporal lobe epilepsy have a previous history of prolonged febrile seizures. The aim of this study
was to determine types of FS, recurrences and occurrence of
epilepsy in our patients with FS.
Method: Retrospective analysis of data of patients with FS
treated at our Neuropediatric Unit and Outpatient Clinic last
20 years was done.
Results: 880 patients had FS: simple 81.1% and complex
19.8%. Thirty-three children had subsequent non-febrile seizures. Epilepsy occurred in 23 children following FS after
1–5 years: in 5 idiopathic generalised; in 7 partial idiopathic or
criptogenic extratemporal and in 2 temporal; in 3 symptomatic par tial. Af ter FS two chil dren had ep i lep tic encephalopathy; 2 had syndromes with molecular abnormalities;
1 had Fahr disease. One girl presented for the first time with
febrile status epilepticus had Sturge Weber syndrome. Between 23 children with epilepsy, previously 13 had one and
10 had complex FS. Betwen 23 children with epilepsy, good
control of epilepsy had 7 children; normal intellectual development had 6 children.
Conclusion: Reviewing our 880 patients with FS, 2.6% developed epilepsy. Between them 2 children had refractory idiopathic temporal lobe epilepsy. Hippocampal sclerosis was
not found in our children with FK and subsequent epilepsy.
SEIZURES AND VALUE OF EEG
INVESTIGATIONS IN ASPHYXIATED TERM
NEWBORN INFANTS
Ulvi Vaher, Aita Napa, Tiina Talvik
Neonatal seizures are markers for perinatal brain damage and
has been correlated with later neurological sequelae. Birth asphyxia is a major cause of perinatally acquired brain injury in
full-term infants. A characteristic feature of neonatal seizures
is the phenomena of electroclinical dissociation: seizures can
be electro-clinical, electrographic (subclinical) or clinical only.
The incidence of seizures in infants born at term is 1.5–3.0 per
1000 live births. The incidence of electrographic seizures is
unknown. There is now evidence that electrographic-only seizures have a similar impact long-term outcome as electroclinical seizures. The prognostic value of electroencephalography (EEG) in full-term infants with hypoxic ischemic encephalopathy (HIE) has been well documented. The aim of the
study was to evaluate the presence of neonatal seizures and
EEG background activity in asphyxiated term infants. Patients
and methods. The study group included 22 term asphyxiated
neonates admitted to Children’s Clinic of Tartu University Hospital (Children’s Intensive Care Department and Department
of Neonatology). 17/22 were born in severe and 5/22 in moderate asphyxia. In 18/22 of them developed HIE (8/18 mild,
7/18 moderate, 3/18 severe). 9/22 were diagnosed clinical
features of neonatal seizures. First EEG was recorded on the
1.–4. day of life. Electroclinical association between clinical
seizures and EEG findings was established in 6/9. 3/9 newborns had normal EEG features (recording was done at day
2–4). In 3/22 neonates without clinical seizures 2 babies had
electrographic seizures and one baby had moderate abnormalities in background activity. EEG in these babies was done
in 5 day of life 2/3 and at the first day of life 1/3.
Conclusions: 1) in all neonates born in asphyxia EEG recording is needed for evaluation of seizures and background activity; 2) the EEG recording should be done as early as the first
day of life; 3) for prognostic purposes serial recordings are
needed.
NEURODEVELOPMENTAL OUTCOME OF
NEONATAL SEIZURES
Jurate Grigoniene, Dovile Rackauskaite,
Milda Endziniene, Nerija Vaiciene
Objective. To determine neurological outcome of neonatal
seizures and factors that may influence it.
Methods. We have retrospectively analyzed 76 cases of neonatal seizures at the Department of Neonatology of Kaunas
S27
Medical University Hospital within 2000–2003. As the second
step questionnaires were sent by post to the parents of children who have experienced seizures in the neonatal period.
Results. Response rate was 47%. Twenty children (55%) of
2–5 years old had normal development and no seizures,
2 children have died. Fourteen children (39%) had abnormal
development: 9 (25%) had cerebral palsy, 4 – language disorder, 1 – mixed developmental disorder. Seven children (19%)
had epilepsy and were on antiepileptic drugs, four of them
had also cerebral palsy, one – language disorder. Even six
children with epilepsy had abnormalities on ultrasonography
(US), performed during neonatal period. There was no significant difference in the neonatal neurological status between
children who later developed seizures and those who did not.
Only 6 children had electroencephalography (EEG) performed in neonatal period, so the information for the analysis
was insufficient. All children with developmental disorders
had abnormalities on US in neonatal period; 36% children
with normal development had changes in US (p<0.01).
Twenty-six children (74.3%) experienced seizures during the
first two days of life, and impaired development or cerebral
palsy were more common in this group to compare with those
who experienced seizures later (p<0.05).
Conclusions. Seizures during the first days of life were related to impaired neurological development. Neonates who
experienced seizures but had no abnormalities on US had
more favourable outcome. EEG would be helpful to predict
prognosis of neonatal seizures.
TREATMENT OF SPASTICITY WITH
BOTULINUM TOXIN A IN CEREBRAL PALSY:
THREE YEARS EXPERIENCE AT KAUNAS
UNIVERSITY HOSPITAL
Svajune Gradeckiene 1, Danguole Ostrauskiene 1,
Lina Norkiene 2, Asta Kutraite 1, Milda Endziniene 1,
Nerija Vaiciene 1
1
Department of Neurology, Kaunas University of Medicine,
Kaunas, Lithuania;
2
Department of Rehabilitation, Kaunas University Hospital,
Kaunas, Lithuania.
Background: Treatment of children with cerebral palsy with
Botulinum toxin A was introduced at our department in 2004.
The aim of the study was to evaluate the efficacy of the treatment on the spasticity of the lower limbs.
Method: We have analysed data of 49 children with cerebral
palsy between 2 and 9 years of age. The scores of spasticity
(Ashwort scale), gait cycle analysis, foot dorsiflexion and the
level of Gross Motor Function Classification System (GMFCS)
were assessed before injection and one month after each injection. The number of injections varied from 1 to 6 for the
same patient. Injections were performed in gastrocnemius,
soleus and adductor muscles. Statistical analysis was performed using SPSS program package, significance assumed
at p < 0.05.
Results: After Botulinum toxin A injections 59.4% of the patients have reached higher functional level according to
GMFCS. It was more significant in patients who had lower
baseline spasticity. Visits and injections at regular intervals as
well as cast wearing significantly improved GMFCS functional
level and gait to compare with irregular ones. Higher number
of injections was related with increased dorsiflexion of foot.
Conclusion: Lower spasticity at baseline, regularity and number of injections were the main factors that influenced more
favourable treatment outcome of spasticity in children with cerebral palsy.
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PHYSICAL CHARACTERISTICS OF
UNAFFECTED LIMBS OF CEREBRAL
PALSIED CHILDREN
Naile Guney 1, Ugur Cavlak 2
1
Physical Therapy Practioner at a Private Rehab. Center, Denizli,
Turkey;
2
Pamukkale University, Turkey
Background: The purpose of this study is to evaluate physical characteristics and functional level of the unaffected side
of the hemiplegic cerebral palsied (HCP) children and to compare with healthy subjects.
Method: A total of twenty-five children with HCP aged 6 and
12 years (mean age; 9.16±1.92 years) who were attending local rehabilitation centers in Denizli and Burdur were included
in the study and were compared with those of matched
25 healthy children among the same age group. Before the
testing; the demographics belonging to the sample such as
age, gender, weight, height and antropometric measurements
(AM) were recorded. Body mass index score, grip strength,
skinfold caliper measurements (SCM), upper extremity (M.Biceps Brachii, M.Triceps Brachii) and lower extremity (M.Quadriceps Femoris, Hamsring Muscles) muscles strenght were
examined. To measure functional level of the subjects; balance tests (eyes open and closed, one leg standing), half
squat performance and one leg-heel raise test were used.
Results: No statistical significant differences regarding AM
were found between the groups. (p=0.05). Extremity disparency measurements, except for tight, also showed no significant
difference between both groups (p=0.05). The same results
were observed for SCM (p=0.05). While there were no significant differences regarding upper and lower extremities muscles strengths between both groups (p=0.05), a significant difference regarding handgrip strength score in favor of healthy
subjects was found (p=0.05). When the balance, half squat
and one leg-heel raise tests score were analyzed, the results
showed that the children with HCP had lower scores in all functional tests than those of matched healthy subjects (p=0.05).
Conclusion: The results of this study show even though the
children with HCP had the same physical characteristics in
unaffected side like healthy subjects, they had lower functional level than that of matched healthy children. Thus, physical therapists should evaluate both physical characteristics
and functional level of the children with HCP.
MUSCULAR PRESENTATION OF INHERITED
DISORDERS OF MITOCHONDRIAL
METABOLISM IN CHILDREN
Birute Skerliene, Jura Tuleviciene, Marija Jakutovic,
Donatas Petroska
Vilnius University, Vilnius, Lithuania
Muscle pathology is a common presentation of inherited disorders of mitochondrial metabolism (IDMM) – respiratory
chain deficiencies (RCD) and fatty acid oxidation defects
(FAOD). It can manifest as the fatal infantile myopathy with severe progressive and generalized weakness, respiratory distress and lactic acidosis, or as myopathy with hypotonia and
progressive weakness of the limbs, intolerance of exertion
and muscle atrophy in the cases of RCD. Myalgia or myoglobinuria are occasionally observed in RCD, whereas recurrent acute rhabdomyolysis (AR) is characteristic of FAOD.
Pathomorphological alterations are usually helpful to sustain
the suspicion of RCD, whereas biochemical investigations of
blood and urine are necessary for FAOD diagnostics. Here we
present the peculiarities of the muscular presentation in the
patients with the different IDMM. Patient 1: Kearns Sayre syndrome in 13 years old boy with ptosis, chronic progressive external ophthalmoplegia, muscle hypotrophy, growth retardation, decreased higher mental function, hystochemical alterations of the muscle tissue. Patient 2: myopathy, peripheral
neuropathy, intestinal pseudoobstruction, lactic acidosis and
pathomorphological alterations of the muscle tissue in
1 month old boy. Patient 3: Barth syndrome in 8 years old boy
with 3-methylglutaconic aciduria, myopathy, cardiomyopathy,
cyclic neutropenia, episodes of hypoglycemia, severe Reye
syndrome-like episode with coma and subsequent severe
neurological deficiency, CT abnormalities. Patient 4: longchain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
in 8 years old girl with the recurrent episodes of metabolic
encephalopathy in infancy and recurrent episodes of AR
since the age of 3 years with characteristic dynamics of
creatine kinase (CK) and other sarcoplasmic enzyme (SE) activity. Patient 5: 12 year old girl with short-chain coenzyme A
dehydrogenase deficiency and recurrent episodes of AR with
characteristic dynamics of CK and other SE activity.
Conclusion: AR was shown to be characteristic of FAOD,
chronic myopathy of various severities and peculiar pathology of multiple organs – of RCD.
JUVENILE ONSET HUNTINGTON DISEASE
Nerija Vaiciene
Huntington disease is distinguished by the triad of dominant
inheritance, choreoathetosis and dementia. The usual age of
onset is in the fourth and fifth decades, but 3–5% of cases begin before the 15th year and some even in early childhood.
We report 15 year-old girl who was consulted at our department for the first time at the age of 11 years and clinically presented with unbalanced gait, impaired speech and increasing
difficulty with fine motor control since the age of 6 years. Initially myotonic dystrophy was suspected based on the symptoms of weakness of facial muscles (opened mouth, slight
ptosis), dysartria, muscle hypotrophy and weakness, low intelligence. Nerve conduction velocity was normal, EMG was
impossible to perform because of patient’s reluctance. Her
mother was healthy; father’s family history was unknown. After
3 years her clinical picture has changed, the most prominent
symptoms became rigidity and bradykinesia. Imaging studies
showed diffuse enlargement of the lateral ventricles. Molecular genetic investigation revealed more than 80 CAG repeats
which confirmed the diagnosis of Huntington disease.
Conclusion. The clinical features of juvenile Huntington disease are often far from classical, giving rise to diagnostic difficulties. Rigidity, bradykinesia, dysarthria are the most prominent features such that Huntington disease should be suspected in children and adolescents presenting with Parkinson-like syndrome.
A CASE OF SEVERE PELIZAEUSMERZBACHER DISEASE CAUSED BY
THE TRIPLICATION OF PLP1 GENE
Milda Endziniene
Pelizeus-Merzbacher disease is an X-linked neurological disorder. Clinical signs include combination of nystagmus,
stridor, hypotonia, spastic quadriparesis, cognitive impairment, ataxia, tremor, and diffuse leukoencephalopathy on
MRI scans. Seizures and perinatal stridor are rare signs, typically seen only in the most severe cases. Growth is poor; de-
velopmental milestones are significantly delayed or never
achieved. As the child ages, limb spasticity usually replaces
the hypotonia, but the child has poor head control and does
not learn to sit unsupported, much less walk. These patients
may survive to the sixth decade of life or longer.
We report a patient who had severe muscle hypotonia,
hyperkinetic hand movements and nystagmus since birth.
Three maternal brothers and patient’s own brother have died
in infancy, they were diagnosed to have had “seizures” or “cerebral palsy”. On the 3rd day of the patient’s life swallowing difficulties occurred. At the age of 4 months he had his first paroxysm: tonic contraction of facial muscles of one side, head
devi a tion to the same side, dystonic posturing of the
extermities, blinking of one eye. The baby seemed not to lose
his consciousness during these episodes, they most likely
were unpleasant as the patient very often he seemed to be unhappy and cried at the moment. These episodes were multiple per day, usually around sleep or when the baby was unhappy (tired, hungry). At the age of 5 years the patient is still
hypotrophic and has severe developmental delay: he is no
speech, no head control or any voluntary purpuseful movements. He smiles and makes joyful sounds as the reaction but
no other verbal or motor contact is possible. Brainstem auditory evoked potentials showed bi lateral impairement of
acoustic nerve, absence of 5th wave. Triplication of PLP1 gene
has been found which may explain the very severe course in
this case.
MELATONIN SECRETION IN CHILDREN:
DEVELOPMENT AND NEUROLOGICAL
DISORDERS
Ruta Praninskiene 1, Aurelija Jucaite 2,
Irena Dumalakiene 3, Mykolas Mauricas 3
1
Vilnius University Children‘s Hospital, Vilnius, Lithuania;
Karolinska Institute, Stockholm, Sweden;
3
Vilnius University Institute of Immunology, Vilnius, Lithuania
2
The endogeous circadian rhythm of melatonin (MLT) secretion is generated by the suprachiasmatic nuclei and entrained
by the light-dark cycle. Children with neurological and psychiatric disorders, such as lesions of the prefrontal cortex, hypothalamus and disorders of the superior cervical ganglia, blindness, Rett and Angelmann syndromes, cerebral palsy, epilepsy, depression, autism, have a wide variety of pathological
changes of the brain which result in fragmentation of sleep. It
is hypothesized that altered regulation of endogenous MLT secretion may be an important factor influencing sleep patterns.
We investigated diurnal MLT secretion in saliva and excretion
of the melatonin metabolite, 6-sulfatoxymelatonin, in typically
developing children. Twenty nine children (17 girls, 12 boys),
from 5.4 to 17.2 years of age, without sleep disorders, with
cephalgia and osteochondrosis, were investiged in the Department of Child Neurology, Vilnius University Children’s
Hospital. Saliva and urine samples were collected during a
24 – hour period every 3 hours. Immunoassay, ELISA kits
(Bühlmann Laboratories AG, Switzerland) were used for the
MLT measurements. Childrens’ sleep was assessed using
standardized questionnaire. Results showed high inter-subject variability of MLT concentrations. Mean daytime concentration of 6-sulfatoxymelatonin was related to age (p=0.03).
There was no effect of gender, age, endocrinological age on
MLT saliva concentrations. Interestingly, peak MLT saliva levels (at 3.00 am) correlated with gestational age (r = 0.36;
p = 0.05). Sleep scores had no relation to MLT levels, but also
correlated with childs gestational age. Thus, preliminary results show that relation between endogenous MLT levels and
sleep patterns is complex and may be influenced by other important factors that determine childs development.
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SLEEP DISORDERS IN CHILDREN WITH
DEVELOPMENTAL DISABILITIES
Poster Presentations
Ruta Samaitiene, Laima Mikulenaite, Liuda Istigeceva
Vilnius University Children Hospital Child Development Center,
Vilnius, Lithuania
Objective: To evaluate the clinical symptoms and ethiology of
sleep disorders of children with developmental disabilities.
Methods: prospective study of a sample of 30 children with
developmental disabilities, treated in Child Development
Center during 2006–2007. Patients with parental complaints
of sleep problems were studied. Several aspects of sleepwake behaviour were analysed using sleep questionnaire,
sleep diary, interview regarding child’s medical and sociofamilial history, psychological difficulties; video recording of
sleep; actigraphy. All patients receive psychological testing.
Results: Subject consisted of children with mental retardation, language and learning disorders, pervasive developmental disorders/autism spectrum disorders, cerebral palsy.
Age range was 1–6 years. We found a wide range of frequently occurring sleep disturbances in their children.
Symptoms of insomnia, parasomnias, and limit-setting sleep
disorder featured prominently. Extrinsic sleep disorders
(limit-setting sleep disorder, sleep-onset association disorder) featured in younger children with language disorders
and disturbed mother-child relationships. Parasomnias and
disruptive night time behaviour were main sleep problems in
children with pervasive developmen tal disorders/autism
spectrum disorders. Sleep related epilepsy was found in
2 cases.
Conclusion: Insomnia and parasomnias are very prevalent
among sleep disorders in children with developmental disabilities. Multiple child and family factors are associated with
sleep disorders.
ENDOVASCULAR TREATMENT OF
INTRACRANIAL ANEURYSMS IN CHILDREN –
FIRST EXPERIENCE
Karlis Kupcs 1, Helmuts Kidikas 1, Igors Aksiks 1,
Svetlana Rudnicka 2, Aleksejs Vinogradovs 1,
Janis Savlovskis 1
1
2
Paula Stradins Clinical University Hospital, Riga, Latvia;
Clinical Hospital Gailezers, Latvia
Objective: to evaluate safety and eficiency of endovascular
treatment of intracranial aneurysms in children under 16 years
of age.
Materials and Methods: During 3 year period 4 patients with
intracranial aneurysms under 16 year of age where treated. In
all cases endovascular embolisation with platinum detachable coils was performed. 3 patients presented with SAH, one
was unruptured aneurysm and patient was complaining
about headache. There where two patients 15 years old, one
patient 12 years old and one 2 years old. Younger child presented with ischemic lesion in basal ganglia and it was dissecting aneurysm. All other 3 patients had a single saccular
aneurysms.
Results: Complete obliteration of aneurysms were achieved
in all cases, there were no intraoperative complications, no
rerupture of aneurysm. All patients where discharged from
hospital without neurological deficite. Follow up MR angio after 6 month untill now is done in 2 cases and showed no
recanalisation of aneurysm.
Conclusions: Endovascular treatment of pediatric intracranial aneurysms is reasonably safe and reliable method.
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PERINATAL NEUROLOGY
AND DISABILITY
VARIATION IN LANGUAGE DEVELOPMENT
AMONG SENSORINEURAL BILATERAL
HEARING IMPAIRDED CHILDREN AFTER
FITTING WITH A HEARING AID OR
COCHLEAR IMPLANT
Sandra Kuske, Ilze Mezinska
SIA Veselibas centrs “Bikernieki”, Riga, Latvia
The number of sensorineural bilateral hearing impairements
who need further adeguate medical, technical, paedagogical,
social and psychological support increasing. Technical support basically depends on the degree of hearing loss. Cochlear implanted children worldwide demonstrates the established method and adeguate technical supply to open up
possibilities for auditory perception and language development for the hearing impaired child. Depending on individual
characteristics at the moment of implantation, the performance of children who receive a cochlear implant varies.
SAMPLE OF COMPLEX
NEUROREHABILITATION FOR CHILD WITH
MULTIPLE DEVELOPMENTAL ANOMALIES
Aija Kaulina, Imants Lanka, Agnese Kretaine,
Zane Tumsevica, Inese Stepko
Center of Interdiciplinary Child Development, Liepaja, Latvia
Background. This paper presents the potential and results of
long–term complex neurorehabilitation in the case of child
with multiple developmental anomalies on the basis of the author’s own experience. Patient – boy with multiple inherited
anom alies – cleft palate, cervi cal area – C1 dysplasie
(anomalie of development), subluxation of C2 after C3, stenosis of spinal tract, missing of upper ribs (1st pair), foot deformities, stigmas in the face area. Following development disorders – psychomotor delay, stereotypic movements, signs of
autism, subluxation of hips and knees, hearing loss (3rd
level).
Method. Methods of assessment – diagnostics by Vojta
method, Munich Developmental diagnostic, video analysis.
Neurorehabilitation was started from early age (4 month) till
7 years – regularly 4 month courses twice pro year. First complex included – therapy by Vojta method, physical therapy –
handling etc., early age speech therapy. From 3.5 year were
included musical therapy and Montessori therapy.
Results. Patients achievements in the 7 years age – walking
with support, full bowel and bladder control, adequate and
purposeful work with methodical materials, interaction with
other children and adults, adequate behaviour in the different
situations in the everyday life. From 7 years child attend special programme of integrative education for children with special needs.
Conclusions. It’s very important to start early age neurorehabilitation for child with multiple development anomalies to
promote quality of integration in the society.
Keywords: multiple development anomalies, early age
neurorehabilitation.
DOWN SYNDROME, EPILEPSY, THE BRAIN
ABNORMALITIES; THREE CLINICAL CASES
Nataliya Smulska, Ludmila Chepiga
Paediatric Hospital, Kiev, Ukraine
Background. Epilepsy in childhood Down syndrome (DS) is
considered to be not much higher then in the general population.
Methods. We had in to the Department of Neurology 3 patients (two boys and one girl). They were born in time, had
typical for DS dysmorphic features and karyotype with regular
trisomy of chromosome 21 (47,XX,+21; 47,XY,+21). The development was delayed. 2 patients had infatile spasm (IS).
The seizures began in the 4.5-th and 8-th months. The third
patient had secondary generalized seizures with post seizures paresis in the right hand and leg along 10–15 minutes.
Epilepsy began in the 1.5 years old.
Results. EEG: first and second patients had hypsarrhythmia;
the third had patterns of symmetrical (with accent in the left
side) spike discharges and irregular slow activity. MRI: all patients had different the brain abnormalities. First – less volume
of the cerebrum, second – periventricular atrophy and third –
narrow and poorly developed superior temporal gyrus, small
cerebellum and pons, agenesia corpus collosum. Then we
began treatment (our patients have been antiepileptic drags –
valproic acid and carbamasepin) the seizures were stopped.
Conclusions. The children with DS can have the different
type of seizures and many brain abnormalities. Abnormalities
are present in the brain of persons with DS, and together with
abnormal neuronal excitability they may lead to increased seizure susceptibility.
OUTCOMES OF INTRAVENTRICULAR
HEMORRHAGE IN PRETERM INFANTS
Milda Dambrauskiene 1, Kristina Zaveckiene 1,
Rugile Pilviniene 2
1
Kaunas University Hospital, Department of Pediatric Diseases,
Kaunas, Lithuania;
2
Kaunas University Hospital, Department of Neonatology,
Kaunas, Lithuania
Background. Intraventricular hemorrhage (IVH) is the most
common brain injury in preterm infants that can have significant long-term effects on children’s development therefore it
is important to carefully monitor the neurologic outcome of
these infants. The aim of our study was to assess the neurodevelopmental outcome in preterm infants with IVH.
Method. The retrospective analysis of medical records of 134
preterm infants with cranial ultrasound abnormalities was performed. These infants were under observation at follow-up
department until 12 months of corrected age.
Results. Their mean gestational age was 29.31 weeks (range
23–36), mean birth weight 1398 g (range 603–3480). Ultrasound examination in the neonatal period revealed, that isolated IVH grade I–II accounted for 22.4% and in combination
with periventricular leukomalacia (PVL) – 9% of cases, isolated IVH grade III-IV – 6.7% and in combination with PVL –
2.2%, PVL alone – 35.8%. During infancy, ventricular dilatation was found in 20% (5/24) of infants with IVH grade I,
44% (8/18) with IVH grade II and 25% (3/12) with IVH grade
III–IV. Posthemorrhagic hydrocephalus was detected in
11% (2/18) of infants with IVH grade II and 75% (9/12) of infants with IVH grade III–IV (p<0.05), 5 of them (1 with IVH
grade II and 4 with IVH grade III–IV) underwent a ventriculoperitoneal shunting. There were statistically significant differences in the outcomes among the groups with different
grades of IVH. While 71.4% of infants with IVH grade I–II were
neurologically unimpaired, only 3 (25%) infants with IVH
grade III–IV were normal, 5 (42%) patients de veloped
psychomotor retardation, and cerebral palsy was diagnosed
in 4 (33%) cases. Conclusions. Infants with IVH grade III–IV
have poorer neurodevelopmental outcome compared to infants with IVH grade I–II. Long-term follow-up is necessary for
children with IVH to detect their disabilities and to design appropriate therapeutic intervention.
A COMPARISON OF QUALITY OF LIFE OF
MOTHERS WITH CHILDREN WITH/WITHOUT
DISABILITIES: A SAMPLE BASED ON
TURKISH POPULATION
Ugur Cavlak 1, Erdogan Kavlak 1, Ela Tarakci 2,
Arzu Razak Ozdincler 3, Hakan Uysal 4,
Suleyman Gursoy 1
1
Pamukkale University, Denizli, Turkey;
Hacettepe University, Turkey;
3
Istanbul University, Turkey;
4
Eskisehir Osmangazi University, Turkey
2
Background: The aim of this study was to compare quality of
life (QOL) of mothers of children with disabilities with mothers
of healthy children.
Method: Five hundred and eighteen mothers in the Western
of Turkey region completed the Short Form Health Survey
(SF-36). The mothers with children 8 to 312 months of age
(190 mothers with physically disabled children, 182 mothers
with intelligent disabilities children, and 146 mothers with
healthy children participated in the study. The mean age of
the mothers was 33.89±7,2 years (ranges: 18–68 years).
Results: The motor developmental level of the children with
disabilities described as follow; 43 apedal (11.5%), 87 quadripedal (23.3%), and 242 bipedal (65%). While significant differences among mothers in the following domains; “role limitations due to physical health”, “energy and fatigue”, “emotional well being”, and “general health” were found, no significant differences were detected concerning “physical functioning”, “role limitation due to emotional problems”, “social
functioning”, and “pain”. These results show that the mothers
with physical disabled children had fewer scores of the SF-36.
When the mothers compared based on the motor developmental level of the children, significant differences were found
in domains of the SF-36 Survey such as; “energy-fatigue”,
“emotional well being”, and “general health”. Namely, the
mothers with children with disabilities at apedal level had
fewer scores of the SF-36 compared with mothers with children at bipedal or quadripedal level.
Conclusion: Having a child with disabilities decreases QOL
of the Turkish mothers. Also, the children at apedal level much
more affect the QOL of the mothers than the children at
bipedal or quadripedal level. The mothers with healthy children had better scores in domains of the SF-36 compared to
the mothers with disabled children.
COGNITIVE OUTCOME OF CHILDREN WITH
STROKE: A PROSPECTIVE STUDY
Margus Ennok 1, Anneli Kolk 2, Rael Laugesaar 2
1
2
Neurology Clinic of Tartu University Hospital, Tartu, Estonia;
Background: Stroke in children is a rare occurrence that can
significantly influence the development and cognitive maturation of affected children. Previous studies on different cognitive functions have given various results but have also shown
S31
more impaired performance of children with neonatal stroke
(NS) compared with childhood stroke (CS) subjects. Not
much is known about long-term changes in different cognitive
functions. Our aim was to study cognitive profile and its
change in children with stroke over the course of a 2-year period.
Method: 20 children (13 with NS and 7 with CS) were tested
on two occasions with NEPSY test battery (mean intertest interval was 2.29 years). NS children had a mean age of 6.86 on
first and 8.64 on second testing, CS children had a mean age
of 8.21 on first and 10.04 on second testing. Their cognitive
performance was compared with groups of age and sexmatched control subjects from regular public kindergartens
and schools.
Results: On the first testing children with stroke had impairments in sensorimotor, visuospatial, language, memory and
attention functions. NS children had a lower performance in
visuospatial, sensorimotor and language skills while CS subjects had more marked memory problems. Most of these deficits were also revealed on second testing, showing impaired
performance in the same subtests in sensorimotor, visuospatial, language, memory and attention domain. Difficulties
in attentional inhibition became more marked over time. Significant improvement was noted in only some subtests. CS
children improved on some memory tests while NS children
improved in spatial construction task.
Conclusions: Stroke in children leads to significant cognitive
decline. Children with NS had more severe cognitive impairment than children with CS. Cognitive deficits seem to be persistent and children make only a limited recovery over a
2-year follow-up period.
THE EFFECTS OF PHYSICAL AND SOCIAL
HEALTH COMPONENTS ON QUALITY OF
LIFE IN TURKISH MOTHERS WITH DISABLED
CHILDREN
Ugur Cavlak 1, Erdogan Kavlak 1, Suleyman Gursoy 1,
Ela Tarakci 2, Arzu Razak Ozdincler 3, Hakan Uysal 4
1
Pamukkale University, Denizli, Turkey;
Hacettepe University, Turkey;
Istanbul University, Turkey;
4
Eskisehir Osmangazi University, Turkey
2
3
Background: Parenting children who are physically or intellectually impaired is known to be linked to the impaired wellbeing of the parents themselves. Parents face a number of
common tasks in managing their child’s afflicted health. However, there is still little available data on quality of life (QOL) in
parents of disabled children in Turkey. This cross-sectional
study was conducted to describe the effects of some variables, which would interfere with the general health, on QOL
of mothers with disabled children (MDC).
Method: Three hundred and seventy two MDC living in the
Western of Turkey region with a mean age of 34.73±7.5
years completed The Short Form Health Survey (SF-36). All
gave their informed consent for participating to the study. We
also recorded demographics of the MDC. The effects of
10 variables such as; motor developmental level of children,
low back pain (LBP), gender of child, clinical type of disability
of child, exercise habit of mother, educational level of
mother (ELM), occupation of mother, involved extremities of
child (IEC), age of mother (AM), and parity on four domains
of the SF-36 including, general health (GH), physical functioning (PF), emotional well being (EWB), and social functioning (SF) were analyzed using backward multiple linear regression.
S32
Results: LBP and ELM were found as common significant
variables affecting the QOL of MDC regarding the following
domains; GH, PF, and EWB. However, LBP and ELM were not
found as significant variables for the SF domain. IEC and AM
were found as significant variables for the SF domain.
Conclusion: In our context, we found that LBP is the most
common significant factor affecting QOL of the MDC in the
sample. Since, MDC have to adapt to new roles, reorganize
their lives and cope with care, parents with disabled children,
especially mothers may benefit from an intensive family competence programme.
EVALUATION OF SPASTICITY AND
TREATMENT SELECTION IN CHILDREN WITH
CEREBRAL PALSY
Svajune Gradeckiene, Inga Siurnaite, Jolita Silanskaite,
Milda Endziniene, Nerija Vaiciene
The management of spacticity and related motor disorders includes non-pharmacological, pharmacological and surgical
methods. Long term management of spasticity in cerebral
palsy (CP) should be based on objective criteria such as
child’s age, rating scales for spasticity, gross motor function
and gait, goniometry, etc. Unfortunately, these criteria are not
sufficiently applied not only in primary care but in specialized
centers as well. Aim of the study was to evaluate the criteria
used for the selection of spasticity treatment modalities and
the control of their efficacy.
Methods. The analysis of medical records of 79 patients from
1 to 17 years old with different spastic forms of CP was performed in four health care institutions of two cities in Lithuania.
Results. Spasticity according to the Ashwort scale was measured just in 4 cases, gross motor function in 1. Oral muscle
relaxants were administered to 39.2% of the patients, in most
cases episodically, for less than 1 month. Effectiveness of
muscle relaxants was not documented in 74.2% cases; in
25.8% of them the subjective opinion was given by parents or
doctors. Splints were applied in 54.8% of cases. Botulinum
toxin – A was used in 11 cases; in 8 of them injections were
started at the age of 2–3 years but continued only in 4 patients. Objective measurements of spasticity were performed
only in 1 case in this group. Orthopedic surgery was performed to 36.7%, the average age of the first operation was
6.4 years.
Conclusions. The monitoring of spasticity and related motor functions as well as the choice and evaluation of treatment modalities are based on no objective criteria in most
cases.
QUANTITATIVE ANALYSIS OF GAIT
PATTERNS IN CEREBRAL PALSY
Zane Pavare 1, T. Kravchenko 1, O. Kudrjasovs 1,
Zigrida Krumina 2, Anita Vetra 2
1
Gait laboratory of Rehabilitation Institute Riga Stradins
University, Riga, Latvia;
2
National rehabilitation center ‘Vaivari’, Jurmala, Latvia
Background. In this study we propose a quantitative analysis
based on tree-dimensional gait analysis in order to evaluate
visible impairments in the major joints of the CP children with
spastic hemiplegia.
Method. Gait analysis was preformed on 15 CP children. 9 of
those were females and 6 were males within the age range of
5 till 18. The clusters of 19 mm spherical retroreflective markers were placed bilaterally on the thighs and shanks lateral
surfaces. In such way the motion range, joint moment and
muscle energy was determined in three dimensions simultaneously in all of the patient’s largest lower extremity joints by
using a six Pro Reflex systems infrared video cameras of
240 Hz, AMTI force plate, 16 channel Delsys electromyograph. Joint kinematics and temporal parameters were calculated using Visual3D motion software.
Results. The basic divisions of the gait cycle into stance and
swing phase were normal. Changes were established in
stance division: decreased single limb support in both legs,
on the effected side for 5%, the unaffected side – 3%. During
initial limb support there was increased plantarflexion moment at the ankle joint, decreased extension moment at the
knee and as a compensation increased extension moment
and power generation at the unaffected hip joint. There is a
decreased ankle plantarflexion moment thereby resulting in
an increased ankle inversion moment on the unaffected limb
at the terminal single limb support.
Conclusion. In CP children with spastic hemiplegia deviations of the stance phase and kinetic data were established in
both legs. Decreased initial double limb support is associated
with difficulties to load response, that require increased extension moment and power generation at the unaffected hip
joint, decreased single limb support of the spastic side related
with loss of stability, decreased terminal limb support – with
difficulties to elevate the leg.
EPILEPSY
TREATMENT OF INTRACTABLE EPILEPSY IN
CHILDREN USING VAGUS NERVE
STIMULATION: ALTERNATIVE MEDICINAL
APPROACH
Anit Singh, Rajakrishnan V., Krishna Dalal,
Manjiri Tripathi
All India Institute of Medical Sciences, New Delhi, India
Purpose: This study aims at managing patients suffering from
intractable epilepsy by applying indirect vagus nerve stimulation (indirect VNS) through reflexology.
Method: This is a 3 years randomized two-arm pilot study.
The group in arm one is treated with pharmacological drugs,
reflexology therapy and indirect VNS. The second arm comprising the control group continue on anti-epileptic drug therapy. Age group 3yrs to 45yrs, who had intractable epilepsy for
at least 3yrs, met the inclusion criteria.
Result: A total of 27 patients have been admitted to the study
till March 2006. (Sample size of study group =13 and sample
size of control group =14). The response of the patients to the
therapy depended on good compliance. 80% of the patients
showed a positive response with regards to reduction in seizure frequency, duration of ictal phase and improvement in
behavioral patterns of patients, at the end of 4 months monitoring period.
Conclusion: This is an ongoing study and the patients will be
monitored for a period of 3 years. Unique response with Indirect VNS reveals the effectiveness of this technique in managing refractory epileptic patients with least side effect and
cost, but with similar results as obtained in an expensive procedure of direct vagus nerve stimulation. Hence this complimentary medicine maintains uninterrupted personal and family lives.
VAGUS NERVE STIMULATION (VNS) IN
CHILDREN: TEN YEARS EXPERIENCE
Jorgen Alving 1, Hanne Nielsen 1, Kern Olofsson 2
1
2
Danish Epilepsy Center, Dianalind, Denmark;
Department of pediatrics, Roskilde Hospital, Denmark
Aim of study. Tto evaluate the short- and long-term efficacy of
VNS in children with intractable epilepsy. Effect parameters
seizure reduction adverse reactions positive effects apart
from seizure reduction.
Patients. 25 children under age 18, all with difficult-to treat epilepsy, followed from 1996–2006. Seven were excluded from
surgery after pre-operative work-up. All were mentally retarded, half of them severely. Epilepsy syndromes 17 localisation-related 8 generalised. Number of AEDs tried before VNS:
6–10. Four also had tried the ketogenic diet.
Results. Seizure response. Seizure reduction by 50% or
more: after 1 year: 6/25, after 2 years: 7/19, after 3 years: 7/15.
One child died during follow-up. Failures in 2 children, VNS
was switched off, and in another two, the stimulator as removed. Adverse reactions after 1 year (N = 25): voice alteration (7), increased seizure frequency (6), coughing (5),
dyspnoea (3), pain at stimulation site (2), non-convulsive status (2), insomnia (2). Positive effects after 1 year (N = 25):
Better mood (15), Improved attention (14), Successful aborting seizures (magnet) (7), shortened post-ictal phase (6).
Conclusion. VNS is a safe, well-tolerated and effective treatment option for children with intractable epilepsy. The effect
seems to be sustained over a prolonged period, and in accordance with previous findings in adults, to increase over time.
The high frequency of positive effects on mood and attention
is noteworthy.
SLEEP DISTURBANCES IN CHILDREN WITH
EPILEPSY
Ruta Praninskiene 1, Danute Vysniauskiene 1,
Jura Tuleviciene 1, Olga Kutuzova 1,
Genute Markvaldiene 1, Aurelija Jucaite 2
1
University Children’s Hospital, Vilnius, Lithuania;
Karolinska Institutet, Institute of Woman and Child Health,
Stockholm, Sweden
2
Introduction: Attention, learning problems in children with
epilepsy can be attributed to their seizures as well as concomitant sleep problems. Thus, understanding of sleep disturbances (SD) in children with epilepsy may help to improve
management of their neurocognitive deficits. The aim of the
present study was to investigate the pattern of SD among children with epilepsy.
Method: 197 children with epilepsy and their parents were
asked to fill out the questionnaire for the SD among children
(Bruni et al, 1996). The scale of sleep disturbances (SDSC) includes: difficulty in initiating and maintaining sleep (DIMS),
sleep breathing disorders (SBD), arousal disorders (DA),
sleep-wake transition disorders (SWDT), disorders of excessive somnolence (DOES), sleep hyperhydrosis (SHY).
Results: 109 boys and 88 girls, 5.5 to 17.9 years of age participated in the study. The total SDSC scores, DA, SBD, SHY
scores were higher in patients with active epilepsy and generalized seizures (p<0.05). Significantly higher SHY, SBD,
SWDT, DA scores were obtained in the sub-group of children
having just night seizures. Patients with Rolandic epilepsy
have lower scores in DIMS (p=0.06) and DOES (p=0.07).
Children with temporal epilepsy had lower scores in SBD
(p=0.04). Gender effect was observed in DA and DOES
scores (females having higher scores, p<0.02).
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Conclusions: The present study suggests that there is a
close relation between seizures and sleep disorders and it is
dependent on the seizure type and time, on the activity of the
disease. The scale of sleep disturbances may be used to select children with epilepsy for the diagnostic overnight polysomnography test. Identification and treatment of these
problems might improve quality of life of children with epilepsy.
KNOWLEDGE AND ATTITUDE OF THE
STUDENTS TOWARDS EPILEPSY
Giedre Jurkeviciene, Jurgita Vainauskiene,
Milda Endziniene, Nerija Vaiciene, Giedre Gelziniene,
Dovile Rackauskaite, Inga Simaityte
Purpose: to assess knowledge and attitude of medical and
non-medical students towards the patients with epilepsy.
Methods: there were 412 questionnaires filled by 280 (68.0%)
students of Medicine Faculty, Kaunas University of Medicine,
Kaunas, and 132 (32.0%) students of non-medical profile specialization from various universities of Lithuania. Questions
were divided into 2 fields: 1) knowledge in epilepsy, 2) attitude
towards the patients with epilepsy.
Results: 249 (88.9%) medical and 95 (71.0%) non-medical
students answered correctly that epilepsy is the disease of the
brain. Non-medical students (15 (10.6%)) made a mistake
considering epilepsy as a psychiatric disease more frequently
than medical ones (12 (4.3%)), (p=0.01). They also more frequently did not know what ep i lepsy is (ac cord ingly:
14 (10.6%) and 6 (2.1%), p<0.001). All 5 proposed correct
reasons of epilepsy were more frequently chosen by medical
(84; 30.0%) than non-medical students (1; 0.8%), (p<0.001).
Non-medical students (49; 37.1%) marked only one possible
reason of epilepsy more frequently comparing with medical
students (59; 51.1%), (p<0.01) and more frequently did not
know the reasons, which cause epilepsy (accordingly:
47 (35.6%) and 30 (10.7%), p<0.001). All 4 proposed correct
manifestations of epileptic seizure were more frequently
marked by medical (99; 35.3%), comparing with non-medical
students (3; 2.3%), (p<0.001). Non-medical students marked
only one possible correct manifestation of epilepsy seizure
more fre quently than med i cal stu dents (ac cord ingly:
67 (50.8%) and (61; 21.8%), p<0.001). The answers to the
questions reflecting positive or negative attitude towards epilepsy were not statistically different between the groups of
medical and non-medical students.
Conclusions: 1. Medical students have better knowledge of
epilepsy than non-medical ones. 2. Attitude of medical students after neurology and non-medical students did not differ
towards the patients with epilepsy.
CHANGES IN EEG WITH DIFFERENT
PERIODS BETWEEN THE LAST SEIZURE AND
EEG RECORDING IN PATIENT WITH
ROLANDIC EPILEPSY
Giedre Jurkeviciene, Nerija Vaiciene,
Milda Endziniene, Viktoras Saferis
Purpose. To determine the probability of finding and the rate
of centrotemporal spikes (CTS) in waking (WEEG) and sleep
(SEEG) electroencephalograms depending on the time period between the last seizure and the EEG recording in patients with Rolandic epilepsy (RE).
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Methods. We studied the clinical, WEEG and SEEG findings
on 121 patients with RE. These children were examined for RE
and were treated at the Child Neurology Unit of Kaunas University Hospital from 01.10.2001 till 01.10.2003. Depending
on the duration between the last seizure and the date of EEG
recording, the patients were differentiated into 7 groups:
Group 1 – < 1 week, Group 2 – > 1 week – < 1 month,
Group 3 – from 1 to 3 months, Group 4 – from 3 to 6 months,
Group 5 – from 6 months to 1 year, Group 6 – from 1 year to
2 years, and Group 7 – > 2 years.
Results. When the period between the last seizure and the
EEG recording is shorter, the probability of finding CTS in
WEEG as well as in SEEG is higher (WEEG c2 =19.8,
p<0.001; SEEG c2=20.4, p<0.001) than in a case of longer
time period. CTS rate in SEEG was significantly different in
groups of different time period between the last seizure and
the EEG recording (Kruskal-Wallis ANOVA: c2=22.8, df=6,
p=0.001), while CTS rate in WEEG in these groups was not
statistically significantly different.
Conclusion. The longer was the period between the last seizure and the day of EEG recording the lower was the probability of finding centrotemporal spikes in awake and sleep
EEG (p<0.001) and the lower was the centrotemporal spike
rate in sleep EEG (p=0.001) in patients with Rolandic epilepsy.
CENTRAL STIMULANT TREATMENT OF
ADHD/ADD IN CHILDREN WITH EPILEPSY
Lene Sahlholdt, Lene Starup, Kirsten Juul,
Tove Hoffmann, Ditte Jeppesen
Danish Epilepsy Hospital, Dianalund, Denmark
32 children, 9 girls and 23 boys, with epilepsy and ADHD/ADD
were analysed in a retrospective study. Age 6 to 18 (mean
10 years). All received treatment with centralstimulants
(31 methylphenidate, 1 methylphenidate plus modafinil) after
neuropsychological evaluation, e.g. observation at the ward
during admission and possibel testing. 29 were psychological tested, 3 were not testable. Full scale IQ were obtained in
25, IQ 40 to 117 (mean 72), IQ < 80 in 14. Centralstimulant
treatment were introduced at the age of 5 to 15 (mean
8.5 years). 3 patients had ADHD hyperactive type, 11 ADD inattentive type, 13 ADHD combined hyperactive/inattentive
type. Ep i lepsy clas si fi ca tion: 14 idiopatic gen er al ised
(6 GTCS, 6 Absence, 1 JME, 1 MAE), 1 Epilepsy with Eyelid
Myoclonus, 1 Epilepsy with Myoclonoc Absences, 1 Progressive Myoclonic Epilepsy, 2 Encephalopathy with CSWS,
2 Symptomatic multifocal, 5 Symptomatic focal, 2 BECTS,
3 Benign Occipital Epilepsy of Childhood, 1 unclassified.
29 were on antiepileptic drugs when treatment with centralstimulants were introduced. Monotherapy in 15, 2 drugs in 10,
3 drugs in 1, 4 drugs in 1. 22 were seizure free when
centralstimulants were introduced, 7 still had seizures, information about 3 were not valid. There were no reports on relapse of seizures and no reports on incresed seizure frequency after introduction of centralstimulants. Sideeffects of
centralstimulant treatment were reported in 16. The parents
reported significant good effect of the centralstimulant treatment on daily life functioning in 30 children who thus continued the treatment, 1 stopped because of lack of effect, information is uncertain in 1.
Conclusion: Treatment with centralstimulants in children with
epilepsy and ADHD is safe and valuable in relation to the
childrens function in daily life.
LONG-TERM TREATMENT OF COMPLEX
PARTIAL SEIZURES STATUS EPILEPTICUS
WITH INTRAVENOUS MIDAZOLAM: A CASE
REPORT
Edita Kanyte, Jurate Grigoniene, Milda Endziniene,
Nerija Vaiciene
Patients with epilepsy may experience clusters of seizures
sometimes progressing to convulsive or non-convulsive status epilepticus (SE). Refractory SE is defined as a condition
that fails to respond to first- and second-line anticonvulsant
medications. The management of repetitive seizures and refractory SE still lacks clear guidelines, and therapeutic approaches are mainly based on the individual experiences of
treating physicians. We present 2-year-old previously healthy
girl who at the age of 9 months started with frequent partial
seizures with secondary generalization characterized by deviation of the eyes to the right, tonic strain, short clonic jerks of
the limbs and followed by postictal prolonged sleep. VideoEEG showed intensive epileptiform discharges in the left frontal-temporal region. CT and MRI were normal. Seizure control
was achieved for 3 months with valproate and nitrazepam.
Later short seizures manifesting with a cry, tonic strain of
arms, vegetative symptoms, frightful look, impaired consciousness relapsed, and the EEG revealed continuous
spike-slow wave discharges in the left frontal-central-temporal
region and less intensive discharges in the parallel right side.
The additional therapy was disappointing: sultiam, corticosteroids, high doses of topiramate (15 mg/kg/d) failed, carbamazepine induced a worsening and lamotrigine caused the
hypersensitivity syndrome. Eventually seizures in clusters developed that progressed to SE of complex partial seizures.
Whereas the initial treatment of SE with intravenous diazepam, phenobarbital and phenitoin failed, the permanent infusion of midazolam was initiated. The dosage had been increased from 1 to 8.3 microg/kg/min until seizures stoped,
then gradually was reduced and discontinued after 18 days.
The treatment was tolerated very well, neither cardiorespiratory depression nor sedation occured. Only single seizures
have been occuring for the past 6 months. Permanent intravenous midazolam is worth a try when basic anticonvulsant
therapy is not able to protect from SE and classic anticonvulsant medications fail.
PERCEIVED STIGMA IN DRAWINGS BY
CHILDREN WITH EPILEPSY
Gintare Zukauskaite, Milda Endziniene
Children with epilepsy (CWE) may experience difficulties in
social integration which is not easy to identify by verbal communication.
Aims: To identify the peculiarities of self-perception of CWE.
Methods: We analyzed the drawings by 210 CWE and of
270 children with other neurological disorders (control
group), hospitalized at Child Neurology unit, Kaunas University Hospital, 3–7 years and 8–17 years of age. Seven topics
were suggested to all participants: Myself (I am real, Myself as
a symbol), Disease, Epilepsy, Time, Place, Emotion, Activity.
Results: in 70% cases of 3–7 years old CWE warm colors
dominated, in 95% of children of 8–17 years old cold colors
did. In most cases CWE identified ‘Myself’ with the disease,
accepted own disease, but could not accept himself/herself
(clear details of the child, clear face of the disease, projection
of the disease in oneself). CWE projected emotions to oneself
while children without epilepsy projected emotions also towards environment (CWE depicted anger as placed inside,
those without epilepsy represented situational drawing; ‘Unhappy Snail’ lived in the closed off house according to the
most drawings of CWE, to compare with just 30% of the control group). CWE were timorous to compare with the control
group (drawings are more faint, from one line, scarce experiments with intense colors, simple structures), more lonely
(phenomenon of one person, one flower, predominate in the
drawings), diffident (the person drawn is with dropped eyes,
hands, without feet), more static (in drawings of action, environmental object are immobile), feeling overprotection from
his family (in drawings other family members are bigger than
him/her), less interested in environment (background in the
drawings are not filled up, structures are very poor, roughcast,
not painted).
Conclusions: The drawings of CWE reflected perceived
stigma which was especially obvious in the older age group.
GENETIC AND NEURODEGERATIVE
DISORDERS
ENVIRONMENTAL FACTORS IN THE
DEVELOPMENT OF AN ACUTE
RHABDOMYOLYSIS IN THE PATIENT WITH
THE SHORT-CHAIN ACYL-COA DEHYDROGENASE GENE POLYMORPHISM 625 G>A
Birute Skerliene 1, Marija Jakutovic 1,
Donatas Petroska 1, Willy Lehnert 2
1
2
Freiburg University, Germany
Short-chain acyl-CoA dehydrogenase (SCAD) is the first enzyme of the mitochondrial short-chain beta-oxidation spiral
catalyzing the dehydrogenation of C4 and C6 fatty acids. Inborn SCAD deficiency is defined and belongs to the group of
the inherited fatty acid oxidation defects. In addition to pathogenic or inactivating mutations, which are responsible for severe diseases, two polymorphisms have been identified which
are common in the general population. Individuals carrying the
625 G>A polymorphism are at risk of developing symptoms.
Objective: other genetic, cellular and environmental factors
are considered to be involved in reducing the level of catalytic
activity of the variant enzymes below a critical level, leading to
the onset of clinical symptoms.
Material and results. A patient with 625 G>A polymorphism
is presented who experienced episodes of acute rhabdomyolysis since the age of 3.4 years. Investigations for the other genetic and inflammatory muscle diseases were negative. The
last episode of an acute rhabdomyolysis resulted in the lethal
outcome because of ventricular asystoly due to hyperkalemia.
Discussion. A deficiency may either be consistently expressed or related to intermittent cellular stress. Both variant
proteins at higher temperatures may result in insufficient
amounts and activity and thus the development of SCAD deficiency. Reduced pH, high fatty acid oxidation activity may result in accumulation of butyric acid which is cytotoxic.
Ethylmalonic acid is supposed to be responsible for the inhibition of the electron transport chain and creatine kinase activity in human skeletal muscle.
Conclusion: the severe course of the disease in the patient
was perhaps determined by an unusual physical loading, fasting, high weather temperature and other unfavourable conditions.
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DOPA-RESPONSIVE DYSTONIA –
A CASE REPORT
Inga Talvik, Kadi Veri, Tiina Talvik
Dopa-responsive dystonia (DRD), first described in 1976 by
Segawa, is a rare hereditary disorder – progressive dystonia
with diurnal fluctuations. The most common form of dopamine-deficit syndrome is an autosomal-dominant condition
caused by mutation of the gene for guanosine triphosphate
(GTP) cyclohydrolase I. In classical cases the disease manifests in early childhood with dystonia of the lower limbs and
there is dramatic and sustained response to treatment with
levodopa. Several authors believe that DRD is an underdiagnosed condition and frequently misdiagnosed as cerebral palsy – “striatal toe” may be misinterpreted as a positive
Babinski sign. We describe a 11-year-old girl with a 2 year
history of progressive walking difficulties. Diurnal variation
of her symptoms were observed – the gait disturbance became worse during the day and after exercise. Her perinatal
and family history was unremarkable. Clinical examination
revealed brisk tendon reflexes, equinovarus posturing of
the left foot and a contracture of the left ankle. Brain computed tomography scan and lumbosacral magnetic resonance imaging were normal. Twenty-four hours after starting treatment with low dose combined levodopa and carbidopa a dramatic improvement was noted in her dystonic
gait. On follow-up one month later there was barely detectable gait disturbance. The patient continues replacement of
dopamine using oral levodopa 4 mg/kg/day and physiotherapy. Dopa-responsive dystonia should be considered and
empiric treatment with levodopa should be started in any
child who presents with progressive dystonia of unkown etiology.
CLINICALLY CONFIRMED
SCHWARTZ-JAMPEL SYNDROME TYPE I
Birute Skerliene, Danute Vysniauskiene
Vilnius University, Vilnius, Lithuania
Schwartz-Jampel syndrome (SJS) is a very rare inherited disease with a distinctive association of myotonia and chondrodysplasia. The pathophysiological mechanisms of SJS are
only recently started to be recognized. SJS results from mutations in the HSPG2 gene encoding a major component of
basement membranes perlecan, in contrast to the other myotonic disorders which arise from mutations in genes encoding
voltage-gated ion channels. Until recently, very few patients
had been studied genetically.
Objective: a patient with this rare and insufficiently investigated disease is presented. A 5.5 years old girl with the diagnoses of epileptic syndrome and cerebral palsy was admitted to the hospital in order to specify the reasons of the joint
stiffness where after the discovery of the irregularities of the
capital femoral epiphyses and vertebra. Myotonia, blepharospasm, diminished muscle mass, thigh hypertrophy,
hyporeflexia, skeletal dysplasia (high-arched palate, micrognatia, short neck, pectus carinatum, coxa valga, pes planovalgus, kyphoscoliosis, reduced stature), were disclosed.
Myotonia presented as painless tonic spasms or temporary
rigidity of the muscles after an active or passive attempt was
made to move them since the age of 1.5 month. Joint
hypermobility was more characteristic during relaxation of
the muscles except some fixed contractures. ENMG showed
continuous electrical activity and normal nerve conduction.
Illnesses of the mother during pregnancy (influenza and
treatment due to cardiologic complications) were disclosed.
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Coloboma of the iris was found in the mother and obscure
signs of myotonia in the otherwise healthy father. The treatment with phenytoinum was started and is still continued
with the doubtful positive effect till her recent 12 years of
age.
Discussion and conclusions: SJS can be not as rare as misdiagnosed; clinical and genealogical peculiarities were disclosed; molecular genetic study should be performed in order
to contribute to the feather research of this pathology.
ANGELMAN SYNDROME: A CASE REPORT
Svajune Gradeckiene 1, Danielius Serapinas 1,
Milda Endziniene 1, Inga Bucinskiene 2,
Jolanta Jeroch 2, Daiva Urboniene 2, Nerija Vaiciene 1
1
Kaunas University Hospital, Neurology Department, Kaunas,
Lithuania;
2
Kaunas University Hospital, Genetic Counceling Unit, Kaunas,
Lithuania
We report a case of genetically confirmed Angelman syndrome, described in 1965 by Angelman. A 7-year-old girl was
referred to the Pediatric Neurology Unit in March 2006 for consultation about possible treatment with botulinum toxin A for
her cerebral palsy. At the age of 6 years she had generalized
tonic clonic seizures provoked by high fever and considered
as febrile ones. Her clinical examination showed severe mental retardation with marked delay in attainment of motor milestones, paroxysms of inappropriate laughter, blond hair, ocular anomalies, including decreased pigmentation of the
choroid and the iris, the latter resulting in pale blue eyes, jerky
arm movements resembling a puppet gait and hypermetropia – symptoms which clin i cally were char ac ter istic to
Angelman syndrome. Sleep EEG showed high amplitude
2.5–3 Hz spike waves generalized activity, episodic asymmetric delta slow spike waves activity in the right frontal region.
Antiepileptic drug treatment was started. The patient was referred for genetic consultation with suspicion of Angelman
syndrome. Cytogenetic-molecular analysis revealed deletion
of 15q 11 chromosome which is typical for Angelman’s syndrome.
Conclusion: This case demonstrates the successful application of newly implemented cytogenetic molecular investigation at Kaunas University Hospital which helps to detect previously unknown causes of neurological disorders.
INFANTILE GM1 GANGLIOSIDOSIS: FATAL
DISEASE DUE TO INHERITED PROGRESSIVE
NEURONAL DAMAGE
Algirdas Utkus 1, Chandra Ward 2, David Booth 2,
Anthony Fensom 2, Birute Tumiene 1,
Jurate Grigoniene 3, Vaidutis Kucinskas 1
1
Biochemical Genetics Laboratory, Genetics Center, Guy’s
Hospital, UK.;
3
Kaunas University Hospital, Department of Neurology, Kaunas,
Lithuania
2
GM1 gangliosidosis is a lysosomal storage disease caused
by mutations in a GLB1 gene and a deficiency of enzyme
beta-galactosidase. Accumulation of undegraded gangliosides occurs in the neurons, forming typical meganeurites,
and in the visceral organs. Our presented patient was a
1.5 years old female with typical symptoms of infantile GM1
gangliosidosis. She was born to non-consanguineous parents of Ukrainian and Russian descent after an uneventful
pregnancy per sectionem cesaream. Edema in lower ex-
tremities and in the periocular region was observed after delivery. Appetite was good and weight gain was normal. Increased irritability, sensitiveness to sounds and fine horizontal nystagm were noted from birth. From the age of two
months infantile spasms were observed: stiffness, redness in
face and apneic episodes started at the time of crying or exercises and lasted for several seconds. There was developmental regress from very early in infancy: the child had a
head control at the age of 3.5 months but later lost it, smiling
appeared only at 11 months, gaze fixation – at the age of 12
months of age. CT scan of the head region was performed in
Kaunas University of Medicine Clinics: hypoplasia of vermis
cerebelli and secondary vetriculomegaly was observed in it.
Typical symptoms of cherry-red spots were found in ocular
fundi. Phenotype at the age of 1.5 years was also suggestive:
profoundly impaired psychomotor development, almost no
active movements, macrocephaly, coarse facial features,
gingival hyperplasia, no teeth, macroglossia, hypertrichosis,
hepatomegaly, abducted great toes in both feet. Diagnosis of
GM1 gangliosidosis was confirmed in the Genetics Center of
Guy’s Hospital in London: characteristic abnormal bands
were observed after the evaluation of urinary oligosaccharides by thin layer chromatography and grossly diminished
activity of beta-galactosidase was determined in blood leucocytes.
HALLERWORDEN–SPATZ DISEASE: CASE
REPORT
Tatjana Volkova, Nerija Vaiciene
Hallerworden – Spatz disease (HSD) is a rare disorder characterized by progressive extrapyramidal dysfunction and
dementia, first described in 1922 as a form of familial degeneration characterized by iron deposition in the brain. The
usual onset is in late childhood or adolescence. Pathologic
evaluation reveals characteristic rust-brown discoloration of
the globus pallidus and substatia nigra pars reticulata secondary to iron deposition. Clinical manifestation is variable,
usually starting with movement disorders such as rigidity of
extremities, slowness of movements, dystonia, choreoathetosis and tremor. Dysphagia and dementia as well as visual impairment due to optic atrophy or retinal degeneration can be presenting symptom in many cases. No biochemical marker has been found in HSD. MRI pattern is
fairly diagnostic for HSD and has been termed the “tiger’s
eye” sign: bilateral symmetric hyperintence signal in anterior medial globus pallidus with surrounding hypointensity
in the globus pallidus and substantia nigra pars reticulata
on T2-weighted images. We present a boy who came to the
medical attention at the age of 2 months because of motor
delay and hyperactive behaviour since 3 years old. At the
age of 3 years incipi ent atro phy of opti cal nerve and
pigmental retinitis were found. Initial extrapyramidal symptoms started at the age of 7 years and rapidly progressed
with development of painful dystonias and dysphagia followed by dementia. Tests for Wilson’s disease, acanthocytosis, lipoid amino acids were negative. Initially CT and
MRI did not reveal any lesions. The “tiger’s eye” sign on MRI
was defined at the age of 9 years. The attempt to reduce
dystonias with Sinemet, Baclofen and benzodiazepines
were of very limited effectiveness. The patient died at the
age of 10.5 years.
Conclusion: HSD should be suspected in case of rapidly progressing dystonia starting in childhood. The typical MRI pattern in a presence of clinical symptoms is pathognomonic for
HSD.
MISCELLANEOUS
NEUROLOGICAL MANIFESTATIONS OF
PEDIATRIC SYSTEMIC LUPUS
ERYTHEMATOSUS WITH ANTIPHOSPHOLIPID SYNDROM: A CASE REPORT
Asta Judickiene, Ruta Praninskiene, Marija Jakutovic
Systemic lupus erythematosus (SLE) is a chronic, inflamatory
autoimune disorder. It may affect the skin, joints, kidneys,
brain and other organs. The central nervous system (CNS) involvement is a common but rarely discussed feature of pediatric systemic lupus erythematosus, particulary with secondary antiphospholipid syndrome (APS). The antiphospholipid
syndrome is a thrombophilic condition manifested by vascular thrombosis with the presence of antiphospholipid antibodies. The spectrum and severity of CNS manifestations of SLE
varies widely, from stroke or seizures to more subtle, abnormalities in neurocognitive functions, such as attention, memory and learning. Thus, the disease must be diagnosed as
early as possible. We report a case of an 11-year-old boy in
whom disease manifested with hemichorea, seizures (1 episode) and behavioral aberrations. Electroencephalography
showed unspecific discharges in the right hemisphere. Carotid duplex Doppler ultrasonography, transcranial Doppler
ultrasonography and brain computed tomography – without
changes. Brain magnetic resonance imaging showed the features of vasculopathy on both sides of parietal region. Anticardiolipin antibodies and anti-beta-2 glicoprotein-1 antibodies were present. Activated partial thromboplastin time (APTT)
was prolonged. Positive antinuclear antibodies and low levels
of complements C3, C4 were found. In our case the correct diagnosis was made 3 weeks after the disease onset. The patient was treated with prednisolone, cyclophosphamide,
acetylsalicylic acid and sodium valproate. His chorea gradually improved and completely disappeared after 2 months, no
more seizures occurred, behavioral abnormalities partially resolved. APTT decreased to nearly normal time.
Conclusions. SLE is to be considered in the differential diagnosis of many neurological conditions. Early diagnosis of the
disease may improve clinical management and prognosis. A
timely and effective management of SLE and prevention of
complications may be achieved by the early detection of APS.
RELATIONSHIP BETWEEN LYME
BORRELIOSIS AND PERIPHERAL FACIAL
PALSY IN CHILDHOOD
Daiva Savickiene, Ruta Praninskiene,
Irena Narkeviciute, Janina Sinkeviciene,
Danute Vysniauskiene, Jura Tuleviciene
University Children’s Hospital, Vilnius, Lithuania
Introduction. Lyme borreliosis is a multisystemic disorder
characterized by several clinical stages. Peripheral facial
palsy (PFP) may be the only sign of Lyme borreliosis in children. This facial palsy requires the diagnostic differentiation
from Guillain-Barre syndrome, Miller-Fisher syndrome, and
especially idiopathic facial palsy (Bell’s palsy).
The aim of our research was to investigate the main etiopathogenic factors of PFP in children, especially in relation to
Lyme borreliosis.
Methods. The prospective study was performed in the Vilnius
University Children’s Hospital, Department of Neurology. We
have tested 58 cases of children with PFP in the period from
S37
September 2004 year to June 2006 serological for neuroboreliosis enzyme – linked immunosorbent assay (ELISA,
Behring, Germany), and Western blotting (WB).
Results: 32 boys and 26 girls, 1 to 18 years of age participated
in the study. Borreliosis was confirmed in 10 (17%) of patients
(3 boys and 7 girls, from 4 to 17 years of age). PFP was observed in 4 cases on the right side and in 6 – on the left side.
Only 2 of the children and their parients remembered having a
tick bite. All the patients didn’t have history of erythema
migrans. All the children became ill in May – October period.
Conclusions: The present study suggests that B.burgdorferi
as ethiopathogenic factor in children with PFP was confirmed
in 17% of cases. Only 3.4% of patients stated having a tick bite
and nobody has observed erythema migrans.
TICK-BORNE ENCEPHALITIS VIRUS
NEUROINFECTIONS IN CHILDREN
Jurgita Grikiniene, Renata Mazeikaite
Background: Lithuania is endemic region of two most common tick-born diseases – tick-borne encephalitis (TBE) and
Lyme disease. TBE is a serios neuroinfection in adults with
high ratio of neurological sequelae. TBE is believed to be a
benign disease in childhood.
The aim of the study was to eveluate peculiarities of TBE virus
neuroinfections in children.
Methods: we retrospectively analysed case histories of all
children diagnosed wih TBE in Vilnius University Children‘s
Hospital in 2003–2005.
Results: we analysed 16 cases (3 girs and 13 boys), aged
13 ± 3 years. Tick bite was known in 14 cases (88%). After an
incubation period of 11 ± 9 days biphasic course of the disease was in 11 cases (69%). An interval between first and second stages was 10 ± 4 days. The second stage manifested as
acute meningitis. All cases were serologically confirmed: Ig M
antibodies of TBE virus were positive. Ig G antibodies were
pos i tive in 10 chil dren (63%). Blood leucocytosis
(14.5 ± 3.02 × 109/l) and granulocytosis (80.6 ± 5.7%) were
found in 15 case (94%). Elevated ESR (26.2 ± 17.5 mm/h)
and CRB (14.3 ± 16.6 mg/l) were found in 12 cases (75%).
Cerebrospinal fluid (CSF) cytosis was from 54 to 656/µl
(164 ± 154/µl) with a predominance of lymphocytes in
14 cases (88%). CSF proteine was elevated (0.71 ± 0.38 g/l).
14 children (88%) were treated with short course (average –
4 days) of glucocorticoids. The hospitalization lasted from 5 to
12 days. All patients had a benign outcome without lasting
neurological deficits or necessity for rehabilitation.
Conclusion: all studied TBE virus neuroinfections cases presented with isolated meningitis serosa of moderate severity.
None of the children studied had lasting neuro logi cal
sequelae.
A CASE OF CNS GERMINOMA IN AN
11 YEARS OLD GIRL
Liutauras Labanauskas
Kaunas University Hospital, Department of Pediatrics, Lithuania
Germ cell tumors, the most common type of tumor in the pineal region comprise a heterogeneous group of primary brain
tumors arising from pluripotential germinal cell. The most
common is the germinoma, accounting for 40–60% of all
germ cell tumors. This tumor account for 0.4–3.4% of intracranial neoplasm. More than 70% of germ cell tumors present
S38
in 10–13 years. Males are 2–4 times more likely than females
to develop a germ cell tumor, but there is a predilection for
suprasellar tumors to occur in females. An 11 years old girl
came to our clinic with disturbed vision, diabetes insipidus
and lethargy. CT scan observed obstructive hydrocephalus
and tumor in hypothalamus region. Stereotactic biopsy confirmed germinoma. Alpha- fetoprotein and beta-human chorionic gonadotropin were negative. Improvement was achived
by placement of ventriculoperitoneal shunt. Radiotherapy
56 Gy and two courses of BEP (bleomycin, ethoposide, and
cisplatin) were administered to this patient. Afterwards the
mother refused to treat her child because of complications of
chemotherapy. Six months later this girl came with the tumor
within the abdomen 6×5 cm close to the outgoing ventriculoperitoneal shunt. The operation was performed and 5 chemotherapy (BEP) courses were given. Now the girl is in complete
remission with normal vision and second-string therapy for
panhypopituitarism.
ROSAY-DORFMAN DISEASE PRESENTING
AS A TUMOUR OF HYPOTHALAMUS REGION
Liutauras Labanauskas
Rosai-Dorfman disease – RDD (sinus histiocytosis with massive lymphadenopathy) is an idiopathic histiocytic proliferative disease characterized by painless massive cervical
lymphadenopathy, fever, leukocytosis, elevated erythrocyte
sedimentation rate, and polyclonal gammopathy. Involvement of variety of extranodal sites is common – skin, bone,
upper respiratory tract, and salivary glands. Involvement of
the central nervous system (CNS) by Rosai – Dorfman disease is unusual and isolated CNS disease in the absence of
nodal disease is distinctly rare. We present a case of extra
nodal RDD manifesting primarily as diabetes insipidus and tumor in hypothalamus region. The patient was 12 year-old girl
who presented with clinical signs of diabetes insipidus. Serum thyroid hormone and hydrocortisone levels were low.
MRI scan showed a 0.9×0.9×1.0 cm tumor in the hypothalamus region. The patient underwent subtotal resection of the
tumor. Biopsy from a mass in the hypothalamus demonstrated inflammatory proliferation with S100 negative, CD1a
neg a tive, CD 68 pos i tive histiocytes show ing signs of
emperipolesis. Local radiotherapy 12 Gy was given. After
9 month girl is in remission without proliferation of the
disease.
RECURRENT PAIN AND USE OF ANALGESIC
DRUGS AMONG SCHOOLCHILDREN IN
LITHUANIA
Donata Jurgaityte, Laura Brauklyte, Liauda Jasionyte,
Nerija Vaiciene
Background. Recurrent pain is one the most often complaint
of the children. The aim of this work was to investigate prevalence of the recurrent pain among schoolchildren, the most
frequent localizations and the intensity of the pain, provocative factors, interference with daily activities and the use of analgesic drugs.
Method. 456 schoolchildren of 10–18 years old were asked to
fill in the original anonymous questionnaire. All children were
chosen incidentally in 3 different regions of Lithuania.
Results: 83.5% of children indicated that they suffered recurrent and troublesome pain; headache was the most frequent
among them. Pain interfered with the daily activity in 86.8% of
children. The average estimation of the pain was severe for
girls and moderate for boys. Mental exhaustion and emotional stress in school were mostly pointed out as the possible
provocative factors of the pain. Two-thirds (66.4%) of pupils
characterized themselves as sensitive and nervous, 64.3% as
constantly tired and having not enough sleep; 46.4% of children indicated that the recurrent pain lasted more than 1 year,
and 17.8% – more than 2 years. The most of children (33.3%)
indicated frequent recurrence of the pain up to several times
per week. Only 42.2% of children were consulted by doctor
for their pain, 60.1% were using analgesic drugs, only half of
them prescribed by doctor. The most often used analgesics
indicated by 158 children: paracetamol 21.2%, metamizol
natrium 14.6%, 32.9% – the combined tablets of acetylsalicylic acid, paracetamol and caffeine.
DYNAMICS OF CHILDREN
NEUROPHYSIOLOGICAL PATTERNS (EEG,
BERA) DURING BRAIN COMA AFTER
SEVERE TRAUMATIC BRAIN INJURY
Conclusions. More than 4/5 of schoolchildren indicated having recurrent pain, mainly headache, which interfered with
their daily activity. In most cases they were not consulted by a
doctor, most of them were using not licensed for children
analgesics. More awareness about childhood pain problem
is needed in primary care as well as in the society and the
family.
Method: Evaluation of coma using Glasgow coma scale
(GCS); EEG mapping; BERA.
TWO CASES OF CAT SCRATCH DISEASE
ENCEPHALOPATHY PRESENTING WITH
STATUS EPILEPTICUS
Jurgis Strautmanis, Inese Kazaine
Children’s Clinical University Hospital, Riga, Latvia
Background. Cat scratch disease (CSD) is a world wide,
non-epidemic infection caused by the Gram negative bacillus
Bartonella henselae. Typical presentation consists of rash, regional lymphadenopathy and general symptoms (fever, headache, malaise). CNS findings occur in 5–10% of cases and
status epilepticus is the most frequent emergency of CSD
encephalopathy.
Case reports. 9 year old girl was admitted to small regional
hospital at 27.10.2006 with complaints on fever and regional
lymphadenopathy of right hand. The patient was scratched
by kitten a month before admission. 5 days later developed
status of generalized tonic clonic seizures and the patient was
transferred to Childrens Clinical University hospital in Riga.
On admission fever, generalized lymphadenopathy (most
prominent in right axillary region), conjunctivitis and corneal
erosion of eyes, unconsciousness, positive Babinski sign and
meningeal signs were present. Seizures were controlled with
Thiopental. 2 days girl was unconscious, but mental status
gradually improved. Subsequently developed ataxia, tremor
and cogwheel rigidity that disappeared within 2 months on
Amantadine. CSF, MRI was normal and various serological,
bacteriological and virology tests were negative. Indirect fluorescent antibody test to B.hehselae was also negative. Lymph
node biopsy and skin testing wasn’t performed. Two months
later 3 years old girl from the same family was hospitalized
with similar clinical presentation. She had had contact with
the same kit ten. In di rect flu o res cent an ti body test to
B.hehselae was also negative.
Con clu sions. There are no pre vi ous re ports of CSD
encephalopathy in Latvia. Although we couldn’t prove diagnosis by serological tests we suppose that these cases could
be referred to CSD because of combination of history (contact with kitten), clinical presentation (regional lymphadenopathy with status epilepticus), lack of changes on CSF examination and MRI and benign outcome. Negative serology test
could be explained by existence of different B.henselae
serotypes.
Ruta Liesiene 1, Ingrida Uloziene 2, Vytautas Ragaisis 3
1
Kaunas University of Medicine, Kaunas, Lithuania;
Laboratory of Neuroscience, Institute for Biomedical research,
Kaunas University of Medicine, Kaunas, Lithuania;
3
Unit of Children Neurosurgery, Department of Neurosurgery,
2
Objectives: The aim of study was to evaluate and compare
the predictive powers of clinical examination combined with
EEG and studies of BERA results in determining the prognosis in traumatic coma.
Results: total of 43 children with severe acute head trauma
were included in the study. Supratentorial injury was established as most frequent type of injury, affecting in 84% of
cases. Intraventricular, parenchimal, subarachnoidal hemorrhagies were noticed in 72%, brain edema in 91% of cases.
Majority of children (35) survived, 8 were dead. The most frequent mortality (38.5%) was amongst the group of children
with GCS 3 in admission. In 19 patients (49%) during EEG
monitoring were observed constant or intermittent slow wave
activity, low amplitude of arrhythmic activity and local slowing
of activity corresponding to brain damage seen on CT. For
17 patients BERA were elicited. In 4 cases it was abnormal
due to acoustic nerve permeability disturbance at the level of
pontine region. In 13 cases no disturbances of permeability
was monitored from the level of cochlea to intermediate brain,
but consciousness recovered after 19 and 24 days.Clinical recovery of consciousness 1 month after trauma was monitored
in 29 cases, 6 patients remained in coma (14%).Continuation
of clinical data and neurophysiological investigations will supporting analysis of prognostic factors and possible outcomes.
Conclusion: Abnormalities on CT and marked EEG not always had abnormal outcome. Abnormal BERA correlated
with EEG abnormalities and brain lesions seen on CT scan
was followed by duration of coma for more than 1 month.
Based on the present data and a literature review combined
clinical examination with results of EEG and BERA can be
used to establish early prognosis in a child traumatic brain injury.
UNUSUAL CLINICAL PRESENTATION OF
GANGLIONEURINOMA LOCATED IN THE
COSTO-VERTEBRAL JUNCTION
Alla Nechay 1, Tatyana Stetsenko 2, Ludmila Chepiga 1
1
2
Paediatric Hospital No 1, Kiew, Ukraine;
Academy of postgraduate training, Ukraine
A case report of the 3 year old girl with ganglioneurinoma in
the costo–vertebral junction is presented Since aged 2y4m
the girl developed sleepiness, sluggish movements and
constipaption. At the time of presentation her sleep was
deep, she did not awaken on her own. When awake she
could unexpectedly lose her muscle tone and fall down,
sometimes she would laugh without context and lose her
tone at the time of such laughter. She appeared weak with
ptosis, muscular hypotonia and losing the possibility for active movements. Myoclonias were seen during sleep and
awakening. Nevertheless the girl did not lose her interest in
toys nor for studying new things. Symtoms progressed rap-
S39
idly; periodi cally tachycardia and tachypnoea appeared.
Blood count, routine biochemistry and urine were normal.
EEG did not show epileptiform discharges. ECG was normal. Brain MRI did not show any ab nor mal i ties. EMG
showed a myasthenic reaction of Lambert–Eaton type. MRI
of mediastinum revealed a tumor of sympathic ganglia
S40
65–19.3 mm in the left costo-vertebral junction at the level of
D5 – D9, that took the contrast non – homogeneously,
ganglioneurinoma was presumed. The case report demonstrates that hypersomnia with paroxysmal events and my asthenic syndrome may be caused by tumor of mediastinum.
INDEX OF AUTHORS
A
H
Aksiks Igors . . . . . . . . . . . . . . . . . . . . . 30
Alving Jorgen . . . . . . . . . . . . . . . . . . . . 33
Hoffmann Tove . . . . . . . . . . . . . . . . . . . 34
I
B
Baiardi Paola . . . . .
Barbon Galluppi Renza
Basel-Vanagaite Lina .
Beni-Adani Liana . . .
Ben-Sirah Liat . . . . .
Biani Naresh . . . . . .
Boltshauser Eugen . .
Booth David . . . . . .
Brauklyte Laura . . . .
Brumback Roger . . .
Bucinskiene Inga . . .
Istigeceva Liuda . . . . . . . . . . . . . . . . . 18, 30
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26
26
24
19
19
19
24
36
38
26
36
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. . 22-23
28, 31-32
. . . . 26
. . 31, 39
. . . . 19
. . . . 23
. . . . 27
C
Caplan Rochelle . . . .
Cavlak Ugur . . . . . .
Ceci Adriana . . . . . .
Chepiga Ludmila . . .
Constantini Shlomi . .
Covanis Anthanasios .
Cvitanovic Sojat Ljerka
D
Dalal Krishna. . . . . . . . . . . . . . . . . . . . . 33
Dambrauskiene Milda . . . . . . . . . . . . . . . . 31
Dumalakiene Irena . . . . . . . . . . . . . . . . . 29
E
Endziniene Milda . . . . . . . . . . . 27-29, 32, 34-36
Ennok Margus . . . . . . . . . . . . . . . . . . . . 31
F
Fensom Anthony . . . . . . . . . . . . . . . . . . 36
G
Gelziniene Giedre . . . . . . . . . . . . . . . . . . 34
Gjergja Romana . . . . . . . . . . . . . . . . . . . 27
Gradeckiene Svajune . . . . . . . . . . . . 28, 32, 36
Grigoniene Jurate . . . . . . . . . . . . . . 27, 35-36
Grikiniene Jurgita . . . . . . . . . . . . . . . . 27, 38
Grinkeviciute Dovile . . . . . . . . . . . . . . . . . 19
Gumbeleviciene Lingvita . . . . . . . . . . . . . . 20
Guney Naile . . . . . . . . . . . . . . . . . . . . . 28
Gursoy Suleyman . . . . . . . . . . . . . . . . 31-32
S42
J
Jakutovic Marija . .
Jasionyte Liauda. .
Jeppesen Ditte. . .
Jeroch Jolanta . . .
Jovic Nebojsa . . .
Jucaite Aurelija . .
Judickiene Asta . .
Jurgaityte Donata .
Jurkeviciene Giedre
Juul Kirsten . . . .
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28, 35, 37
. . . . 38
. . . . 34
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22, 29, 33
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. . 35
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31-32
. . 39
. . 30
20, 38
22, 31
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. . 36
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. . 30
. . 30
. . 28
. . 33
Labanauskas Liutauras
Lanka Imants . . . . .
Laugesaar Rael . . . .
Lehnert Willy . . . . . .
Leskauskas Darius . .
Liesiene Ruta . . . . .
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38
30
31
35
22
39
Magistris Michel R. . . .
Malenica Masa. . . . . .
Mannamaa Mairi . . . . .
Markvaldiene Genute . .
Matukevicius Algimantas
Mauricas Mykolas . . . .
Mazeikaite Renata . . . .
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18
27
19
33
20
29
38
K
Kanyte Edita . . . .
Kaulina Aija . . . .
Kavlak Erdogan . .
Kazaine Inese . . .
Kidikas Helmuts . .
Kiudeliene Rosita .
Kolk Anneli. . . . .
Kravchenko T. . . .
Kretaine Agnese . .
Krumina Zigrida . .
Kucinskas Vaidutis
Kudrjasovs O. . . .
Kupcs Karlis . . . .
Kuske Sandra . . .
Kutraite Asta . . . .
Kutuzova Olga . . .
L
M
Mezinska Ilze . .
Mikulenaite Laima
Morten Enrico . .
Muhle Hiltrud . .
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18, 30
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Napa Aita . . . . .
Narkeviciute Irena .
Nechay Alla . . . .
Nielsen Hanne . . .
Nikanorova Marina
Norkiene Lina . . .
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27
37
39
33
20
28
Siurnaite Inga . . .
Skerliene Birute . .
Smulska Nataliya .
Sojat Tina . . . . .
Spaapen Leo . . .
Spokiene Indre . .
Stakisaitis Donatas
Starup Lene . . . .
Stephani Ulrich . .
Stepko Inese. . . .
Stetsenko Tatyana.
Strautmanis Jurgis.
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. . . . 32
28, 35-36
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. . . . 27
. . . . 25
. . . . 26
. . . . 26
. . . . 34
. . . . 24
. . . . 30
. . . . 39
. . 21, 39
T
O
Olofsson Kern . . . . . . . . . . . . . . . . . . . . 33
Ostrauskiene Danguole . . . . . . . . . . . . . . . 28
Ozdincler Arzu Razak . . . . . . . . . . . . . . 31-32
P
Pavare Zane . . . . . . . .
Petroska Donatas . . . . .
Pilviniene Rugile . . . . . .
Plioplys Sigita . . . . . . .
Plouin Perrine . . . . . . .
Posada de La Paz Manuel
Praninskiene Ruta . . . . .
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. . . . 32
. . 28, 35
. . . . 31
. . 21, 23
. . . . 23
. . . . 26
29, 33, 37
Talvik Inga . . . . . . . . . . . . . . . . . . . . 19, 36
Talvik Tiina . . . . . . . . . . . . . . . . . . 19, 27, 36
Tarakci Ela . . . . . . . . . . . . . . . . . . . . 31-32
Taruscio Domenica . . . . . . . . . . . . . . . . . 26
Tripathi Manjiri . . . . . . . . . . . . . . . . . . . . 33
Tuleviciene Jura . . . . . . . . . . . . . . . 28, 33, 37
Tumiene Birute . . . . . . . . . . . . . . . . . . . 36
Tumsevica Zane . . . . . . . . . . . . . . . . . . . 30
U
Uloziene Ingrida .
Urboniene Daiva .
Utkus Algirdas . .
Uysal Hakan . . .
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31-32
R
Rackauskaite Dovile .
Ragaisis Vytautas . .
Rajakrishnan V. . . .
Rudnicka Svetlana. .
Rutkauskiene Giedre
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27, 34
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18, 30
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. . 25
. . 36
. . 32
. . 34
. . 33
. . 37
S
Sabol Zlatko . . . .
Saferis Viktoras . .
Sahlholdt Lene. . .
Samaitiene Ruta . .
Savendahl Lars . .
Savickiene Daiva .
Savlovskis Janis . .
Schieppati Arrigo .
Sejersen Thomas .
Serapinas Danielius
Silanskaite Jolita . .
Simaityte Inga . . .
Singh Anit . . . . .
Sinkeviciene Janina
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V
Vaher Ulvi . . . . . . . . . . . . . . . . . . . . . . 27
Vaiciene Nerija . . . . . . . . . . 20, 27-29, 32, 34-38
Vainauskiene Jurgita . . . . . . . . . . . . . . . . 34
van Emde Boas Walter . . . . . . . . . . . . . . . 20
Veri Kadi . . . . . . . . . . . . . . . . . . . . . . . 36
Vetra Anita . . . . . . . . . . . . . . . . . . . . 18, 32
Vinogradovs Aleksejs . . . . . . . . . . . . . . . . 30
Volkova Tatjana . . . . . . . . . . . . . . . . . . . 37
Vollmer Brigitte . . . . . . . . . . . . . . . . . . . 18
von Spiczak Sarah. . . . . . . . . . . . . . . . . . 24
Vysniauskiene Danute . . . . . . . . . . . . 33, 36-37
W
Ward Chandra . . . . . . . . . . . . . . . . . . . . 36
Z
Zaveckiene Kristina . . . . . . . . . . . . . . . . . 31
Zukauskaite Gintare . . . . . . . . . . . . . . . . . 35
S43
S44
LIST OF PARTICIPANTS
LIST OF PARTICIPANTS
(registered up to May 1)
CROATIA
Cvitanovic Sojat Ljerka
ljerka-cvitanovic.sojat@zg.t-com.hr
DENMARK
Alving Jorgen
kfjral@vestamt.dk
Holm Anne Marie
Annemarie.Holm@cyberonics.com
Jaegergaard Jane
jansne@vestamt.dk
Nielsen Hanne
kfhani@vestamt.dk
Nikanorova Marina
kfmank@vestamt.dk
Sahlholdt Lene
kflesa@vestamt.dk
ESTONIA
Ennok Margus
margus.ennok@ut.ee
Kolk Anneli
anneli.kolk@kliinikum.ee
Rein Reet
reet.rein@kliinikum.ee
Seling Ly
ly.seling@novartis.com
Siitan Maia
maias@ph.ee
Tali Reet
reet.tali@kliinikum.ee
Talvik Inga
inga.talvik@kliinikum.ee
Talvik Tina
Tiina.Talvik@kliinikum.ee
Teeaar Anne
anne.teeaar@pak.ee
Vaher Ulvi
ulvi.vaher@kliinikum.ee
FRANCE
Dulac Olivier
o.dulac@nck.aphp.fr
Plouin Perrine
perrine.plouin@nck.aphp.fr
GERMANY
Stephani Ulrich
stephani@pedneuro.uni-kiel.de
GREECE
Covanis Anthanasios
graaepil@otenet.gr
S46
INDIA
Singh Anit
dranitsingh@yahoo.co.in
ISRAEL
Basel-Vanagaite Lina
basel@post.tau.ac.il
Beni-Adani Liana
lianabenia@gmail.com
ITALY
Baiardi Paola
pbaiardi@cvbf.net
Schieppati Arrigo
aschieppati@ospedaliriuniti.bergamo.it
LATVIA
Bingeliene Arina
iriss1206@yahoo.com
Cudere Anna
talsuslimnica@inbox.lv
Jostmane Ilze
ilzeliep@inbox.lv
Kazaine Inese
inesek@one.lv
Kikule Ilga
agnese.x.taurina@gsk.com
Kogane Jekaterina
jekaterina.k@balticom.lv
Kondrate Kristina
dr.kkondrate@inbox.lv
Krauce Marika
Krumina Zigrida
gedina@inbox.lv
Kupcs Karlis
kupcs@hms.lv
Lanka Imants
dr.lanka@apollo.lv
Lindenberga Sarma
sarmali@btv.lv
Madre Sarmite
sarmitemadre@inbox.lv
Meiksane Vizma
vizmameik@inbox.lv
Mezinska Ilze
ilzezerba@inbox.lv
Norite Baiba
bnorite@one.lv
Ozola Sarmite
ozolasarmite@inbox.lv
Pavare Zane
gaitazp@inbox.lv
Rozentals Guntis
dr.rozentals@inbox.lv
Strautmanis Jurgis
strautmanis@e-apollo.lv
Tihomirova Margarita
olden@one.lv
Valge Liga
ligakaukis@inbox.lv
Vetra Anita
vanita@cc.lv
Vitols Egils
evitols@gailes.lv
Voita Valda
LITHUANIA
Abelyte Rasa
rasaabelyte@yahoo.com
Adomaitiene Lione
ladomaitiene@one.lt
Alaune Arvydas
arvydasalaune@yahoo.com
Bakaniene Indre
bakind@gmail.com
Balkaitiene Rita
rita.balkaitiene@zebra.lt
Bandanskyte Ausra
ausra@medcon.lt
Barkauskiene Praurima
praurima@gmail.com
Bieliauskiene Laimute
laimuteb@kaunas.init.lt
Brusokiene Grazina
valerija.tabariene@vuvl.lt
Dambrauskiene Milda
milda_da@yahoo.com
Dolebiene Ruta
r.dolebiene@one.lt
Drulyte Audrone
audra17@gmail.com
Endziniene Milda
endziniene@gmail.com
Gradeckiene Svajune
svajuneg@yahoo.com
Grendaite Birute
birutegrend@splius.lt
Greseviciene Vitalija
v.greseviciene@gmail.com
Grigoniene Jurate
grigoniene.jurate@gmail.com
Grikiniene Jurgita
jurgita.grikiniene@mf.vu.lt
Grinkeviciute Dovile
dovilgrin@hotmail.com
Gumbeleviciene Lingvita
lingvita@yahoo.com
Jaskeviciene Vanda
vandaj@one.lt
Judickiene Asta
giedrasta@takas.lt
Jurgaitiene Ruta
donjurgait@yahoo.com
Jurgaityte Donata
donjurgait@yahoo.com
Jurkeviciene Giedre
gjurkeviciene@yahoo.co.uk
Kaminskiene Aurika
loreta.korsakiene@takas.lt
Kanyte Edita
edita.kanyte@gmail.com
Kiudeliene Rosita
rosikiud@gmail.com
Kuliesiene Ausra
ausrakul@takas.lt
Kumetaitiene Ona
o.kumetaitiene@gmail.com
Kusliene Dalia
vismantas_k@hotmail.com
Leskauskas Darius
dleskauskas@yahoo.com
Liesiene Ruta
ruta_liesiene@yahoo.com
Maciuleviciene Ruta
gerimanta@takas.lt
Mameniskiene Ruta
ruta.mameniskiene@santa.lt
Markvaldiene Gene
gene.mark@one.lt
Maskeliene Vida
karina.kovalenkiene@lit.b2i.net
Maskolenkaite Giedre
mgiedre@gmail.com
Matukevicius Algimantas
algimantas.matukevicius@kmuk.lt
Mikulenaite Laima
Laima.mikulenaite@raida.lt
Mykolaityte Diana
diamy@delfi.lt
Niemciene Vida
nivida@infoseka.lt
Norkiene Lina
linanork@gmail.com
Ostrauskiene Danguole
ostrausk@kaunas.init.lt
Paulikiene Violeta
kauliukaz@gmail.com
Pauza Valius
neurologijosklinika@kmuk.lt
Pavalkiene Snieguole
pavalkienes@yahoo.com
Praninskiene Ruta
ruta.praninskiene@elnet.lt
Priceviciute Renata
nigraaa@one.lt
Rackauskaite Dovile
dovile_rackauskaite@yahoo.com
Ragaisis Vytautas
vragaisis@yahoo.com
Rancelyte Rasa
rasarancelyte@yahoo.com
S47
Rastenyte Daiva
daiva.rastenyte@kmuk.lt
Roviene Daiva
daiva.roviene@mail.lt
Rutkauskiene Giedre
giedre.rutkauskiene@medicina.lt
Samaitiene Ruta
samaitiene@yahoo.com
Savickiene Daiva
ruta.praninskiene@elnet.lt
Savickiene Danute – Regina
sl.vlkrastine@su.lt
Savilovas Algimantas
vaiku.ligonine@takas.lt
Serapinas Danielius
dserapinas@gmail.com
Sereikiene Rima
rima_sereikiene@merck.com
Seskiene Jolanta
egis@boo.lt
Simkoniene Alicija
a.gilyte@gmail.com
Sinkeviciene Nijole
janina.sinkeviciene@gmail.com
Skerliene Birute
Birute.Skerliene@mf.vu.lt
Spokiene Indre
biok@mruni.lt
Strupiene Laimute
laima.strupiene@gmail.com
Tabariene Valerija
valerija.tabariene@vuvl.lt
Tamasauskiene Liudmila
ltamasauskiene@yahoo.com
Utkus Algirdas
autkus@hotmail.com
Vaiciene Nerija
nerija.vaiciene@kmu.lt
Vedeckiene Dalia
vedeckiene@gmail.com
Verkiene Ieva
verkiene@one.lt
Volkova Tatjana
tsdi@ktu.lt
Vysniauskiene Danute
pediatrijos.centras@mf.vu.lt
Zaveckiene Kristina
kristina.zaveckiene@gmail.com
Zeguniene Sigita
artrolita@klaipeda.omnitel.net
Zukauskaite Gintare
eleveje@yahoo.co.uk
SERBIA
Jovic Nebojsa
njjovic@eunet.yu
S48
SWEDEN
Ekberg Gunilla
gunilla.ekberg@gbgsd.se
Jucaite Aurelija
aurelija.jucaite@ki.se
Ljungberg Ann-Christine
annchristine.ljungberg@gbgsd.se
Olsson Ulla
ulla.olsson.613@gbgsd.se
Sejersen Thomas
thomas.sejersen@ki.se
Vollmer Brigitte
brigitte.vollmer@ki.se
SWITZERLAND
Boltshauser Eugen
eugen.boltshauser@bluewin.ch
Magistris Michel R.
michel.magistris@hcuge.ch
THE NETHERLANDS
Spaapen Leo
Leo.Spaapen@GEN.unimaas.nl
van Emde Boas Walter
wvemdeboas@sein.nl
TURKEY
Cavlak Ugur
ucavlak@yahoo.com
Kavlak Erdogan
kavlake@hotmail.com
UKRAINE
Chepiga Ludmila
Efimkin.Andrey@phs.com.ua
Davydova Iuliya
juliadavyd@mail.ru
Nechay Alla
allanechay@ukr.net
Ryslyayeva Viktoriya
Smulska Nataliya
UNITED KINGDOM
Stephenson John BP
john@jbpstephenson.com
USA
Brumback Roger
RogerBrumpack@creighton.edu
Caplan Rochelle
rcaplan@ucla.edu
Plioplys Sigita
splioply@childrensmemorial.org
To the Authors of “Seminars in Neurology”
The Journal “Seminars in Neurology” presents review articles and original research papers on the latest advances in etiopathogenesis, clinics, diagnostics, treatment and prevention of neurological,
neurosurgical and adjacent pathology. It publicizes,
as well, case reports about rare or diagnostically
complicated clinical events, materials of scientific
conferences, and other important scientific information.
Articles are presented in English or in Lithuanian.
Articles already presented in other scientific publications are not accepted to the journal except if they
had been submitted as abstracts.
All the scientific articles submitted for publication
are being reviewed by Editorial board and experts of
particular field of medicine.
Scientific articles are published in the journal
complimentary.
The volume of manuscript (including syllabustext, charts, pictures, images) should not exceed
30 typed pages, and the list of references should not
contain more than 60 quoted sources. It is necessary
to indicate personal address and e-mail of the author
whom Editorial board might contact with, as well, first
and last names, degrees, institutions and offices of
all authors.
The structure of the article: title, names of the
authors; institution (-s), where the research/work has
been performed; summary; keywords, syllabus-text;
references.
Original research paper’s text and summary
consists of preface, objective, methods, results, discussion; conclusions.
The structure of review article is chosen by the
author. It is necessary to indicate that the article is
considered to be a review.
The text of case report includes preface, description of the case (-s), discussion.
All the abbreviations used in the text should be
explained. All the charts are submitted on separate
pages, numerated according their sequence in the
text, include their titles. Illustrations should be compact, suitable for printed reproduction, pictures and
diagrams painted with ink or computer (Corel DRAW
preferred), positives contrast, on glance paper. While
submitting photo images on CD, TIFF size (distinction of at least 200 dpi) is preferred. Each illustration
or image should have its title. The places where the
charts (pictures, diagrams, images) are to be inserted should be marked by the author. The author’s
name, title of the article, numeration and the top of
the image should be indicated on the other side of
each illustration.
List of references includes the sources of literature quoted in the text according to sequence of citation. It should be printed on a separate page in original language.
The citation number of reference is indicated in
square brackets.
EXAMPLE:
References
Article:
1. Brodie MI, Por ter RI. New and po ten tial anticonvulsants. Lancet 1990; 336: 425–6.
2. Corbo M, Nemmi R, Iannaccone S, et al. Peripheral
neuropathy in scleroderma. Clin Neuropathol 1993;
12: 63–7.
Chapter:
3. Biller I. Non-atherosclerotic vasculopathies. In: Biller I,
ed. Stroke in children and young adults. Boston:
Butterworth-Heineman, 1994; 57–81.
Book:
4. Klumbys L. Ûminës galvos smegenø traumos neurofiziologija. V., 1979.
5. Pryse-Phillips W. Companion to clinical neurology.
Boston: Little, Brown, 1995.
The manuscripts are submitted to the Editor-inchief V. Budrys by post or e-mail.
Editorial address:
Vilnius University Hospital Santariðkiø Klinikos
Santariðkiø 2, LT-08661 Vilnius, Lithuania
Tel./fax (370 5) 2365 220
Mob. phone +370 698 44049
e-mail: valma@takas.lt
http://www.neuro.lt

about the con fer ence - Neurologijos seminarai

Transcription

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