Child and developmental neurology

Transcription

138
Posters, Sunday 11 September
Child and developmental neurology
P1205
P1204
NEUROTROPHIC FACTOR BDNF
IN CHILDREN WITH BRAIN
PERINATAL AFFECTATION
A CRITICAL ROLE OF CEREBROSPINAL
FLUID IN FOLATE HANDLING FOR THE
CEREBRAL CORTEX
B. Nash, I. Zulfiqar, J. Miyan
Faculty of Life Sciences, The University of Manchester, UK
Delivery and availability of folate to the developing cerebral
cortex is critical for normal development. A unique folate
handling system operates through the cerebrospinal fluid
(CSF) to supply neural stem cells and developing cerebral
cortex with this critical metabolite. In the hydrocephalic
Texas (H-Tx) rat we have found that obstruction of CSF
flow/drainage, occurring in the early stages of cortical
development, results in a failure to secrete the folate binding
protein, 10-formyl-tetrahydrofolate dehydrogenase (FDH).
This results in a consequential arrest of cell cycle in the
cells of the neuroepithelium. In this study we investigated a
range of human neonatal conditions presenting with fluid
accumulation requiring fluid drainage. CSF was collected
from these patients and was compared to normal samples of
CSF collected by lumbar puncture for routine testing for
suspected meningitis. Western blotting for FDH and other
key folate enzymes show a decrease in these proteins in
hydrocephalus through any cause and age of onset but
normal amounts in conditions where fluid obstruction has
not occurred including spina bifida, encephelocels and
Dandy-Walker variants. Similar to our findings using the rat
model, we believe that the former conditions would obtain
significant benefits from a combination folate therapy that
by-passes the missing/reduced FDH.
E. Hadjiu1, A. Hadjiu2, S. Hadjiu1, I. Ilciuc1, C. Calcii1,
L. Ciobanu1
1Neurology, State Medical and Pharmaceutical University
’N.Testemitsanu’, Chisinau, Moldova, 2Psychology,
University of Reims, France
Objectives: We appreciated the brain-derived neurotrophic
factor (BDNF) in the serum of children with diverse grade
perinatal hypoxic-ischemic injuries (PHIE) in acute and
retrieval periods.
Materials and research methods: 182 children aged from
7 days to 12 months, that suffered from diverse grade
perinatal hypoxic-ischemic injuries of the CNS were
investigated.
Results: Low levels of BDNF were frequently appreciated
at children with severe forms of PHIE that are often
associated with disorders of conscience, convulsive
manifestations, disturbances of muscular and reflex tonus.
Likewise, low levels of BDNF were identified in newborns
presenting pathologic EEG patterns and with neuroimagistic data attributed to cerebral hypoxia-ischemia. Low
values of BDNF confirm the presence of severe injuries of
cerebral tissue by the age of 12 months. BDNF level in
children from the study lot remains decreased in comparison
to those from the control group.
Conclusions: The present study confirms that BDNF is an
important marker of destructive lesional sufferance of
cerebral tissue. Very low levels are preserved in children
with severe motor deficit and at those with recurring
epileptic seizures. It is known that BDNF manages processes
of neurogenesis, when BDNF decreases the latest
decompensate. It is possible that the retention of synthesis
of neurotrophic factors could be one of the most important
causes that stops the nervous cell maturation processes in
children with PHIE. Likewise, low BDNF level (lower than
790pg/ml) has a predictive value for the neuropsychic and
motor retard in children with severe PHIE.
© 2011 EFNS European Journal of Neurology 18 (Suppl. 2), 66–343
P1206
AN MRI AND PROTON SPECTROSCOPY
STUDY OF THE BASAL GANGLIAS IN
CHILDREN WITH AUTISM AND
PSYCHOMOTOR RETARDATION
Q. Lee, Y. Xiao, R. Wu
Medical College of Shantou University, Shantou, China
Background: Autism is a behavioural syndrome defined by
the presence of pervasive social deficits, communication
abnormalities, and restricted, repetitive, and stereo- typed
patterns of behaviours. More and more energy has been put
into the brain research while the knowledge is increasing.
The consensus view is that the abnormalities of ASD mainly
involve in the structure and the network function of the
brain. It is possible to use MRS to detect the change of the
brain in-vivo. Those provide a way to find the change of the
diseases in the early stage and monitor the conversion. Basal
ganglia alterations have been reported in autism and nonautistic psychomotor retardation subjects.
Methods: We used 1-H MRS to study absolute metabolisms/
neurotransmitters concentrations in 11 children with ASD
aged 2-6 years old, 20 children with psychomotor retardation
not suffering ASD aged 1-6 years old. Peaks of
N-acetylaspartate (NAA), choline (Cho), creatine (Cr),
glutamate (Glu), glutamine (Gln) and γ-aminobutyric acid
(γ-GABA) were observed in both groups.
Results: No statistically significant differences between the
autism group and the non-autistic psychomotor retardation
group were found on a 1.5T MRI.
Conclusions: The differences about metabolism/
neurotransmitter concentrations between the autism group
and the psychomotor retardation group were not seen in
1.5T MRS. That may indicate that autism and non-autistic
psychomotor retardation have the same pathological
changes in basal ganglia in microscopic pathological
changes.
P1207
PSYCHOGENIC GAIT DISORDERS IN
CHILDREN: NOT SO RARE!
H. Benrhouma, I. Rebai, I. Kraoua, J. Yacoubi,
A. Rouissi, I. Turki, N. Gouider-Khouja
Department of Child and Adolescent Neurology, National
Institute of Neurology, Tunis, Tunisia
139
acuity). Neurological examination showed variable motor
features: limb pseudo-paralysis (5 cases), hemidystonia
(1 case), lower limb paroxysmal rigidity and clonic
movements of upper and lower limbs (1 case).
Neuropsychological testing revealed psychiatric disorders:
conversion (4 cases), anxiety (2 cases), and depression
(1 case). Brain imaging was normal in 6 patients. All
patients improved with placebo treatment, psychotherapy or
both. Recurrence of gait disorders was noticed in four
patients.
Comments and conclusion: PGD in children are usually
misdiagnosed. It is necessary to analyze clinical features
and outcome to reach a clear diagnosis and adequate
management which requires multifaceted approach.
P1208
VAGUS NERVE STIMULATION (VNS) IN A
PEDIATRIC POPULATION: SURGICAL
TECHNIQUE CONSIDERATIONS IN
YOUNG CHILDREN AND ADOLESCENTS
Y. Awaad
KFMC, Riyadh, Saudi Arabia
During the past decade, vagus nerve stimulation has become
an accepted treatment method for patients with refractory
epilepsy who are not proper candidates for invasive,
potentially curative epilepsy surgery such as lesionectomy
or mesial temporal lobectomy. We aimed to review our
clinical experience with vagus nerve stimulation in a
paediatric and young adolescent population specifically
focusing on the necessary special considerations for these
young patients and on surgical pearls in this age group. This
is a single center study from the Oakwood Hospital Epilepsy
Clinic in which all our consecutively enrolled patients were
followed as a cohort with ongoing concurrent as well as
retrospective review of in-patient and outpatient records. All
patients (n=30) had a technically successful initial
implantation procedure, but 4 (13%) required additional
interventions. This included 6 revisions for mechanical
causes, pectoral dehiscence and explants as well as
re-implantation. Epilepsy control improved remarkably in
20 patients (67%) resulting in an improved quality of life for
the majority of our patients. Appropriate technical
modification and attention to detail is needed in these young
patients to achieve optimal surgical results.
Introduction: Psychogenic gait disorders (PGD) in children
are rare and often difficult to distinguish from organic ones.
Objective: To review clinical characteristics, outcome and
treatment in 7 children with PGD.
Methods: 7 children with PGD were followed-up in our
department (January 2008-December 2010). Medical
history, physical examination, video-taping and management
were analyzed.
Results: 4 females and 3 males (mean age 12 years, mean
age of onset 9.5years, mean follow-up period 11months)
had PGD. 4 cases presented with associated multiple
somatic complaints (pain, paraesthesia and impaired visual
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P1209
P1210
COMPARATIVE ANALYSIS OF BRAIN
DERIVED NEUROTROPHIC FACTOR
(BDNF) LEVELS IN CHILDREN WITH
DIFFERENT GRADE HYPOXIC-ISCHEMIC
AFFECTATIONS OF the CENTRAL
NERVOUS SYSTEM
AGE DEPENDENCE ON EEG CORTICAL
CONNECTIVITY IN CHILDREN WITH
ATTENTION DEFICIT HYPERACTIVITY
DISORDER
A. Hadjiu1, E. Hadjiu2, S. Hadjiu2, I. Ilciuc3, C. Calcii3,
L. Ciobanu3
Psychology, University of Reims, France, 2Neurology,
Neuropediatrics, State Medical and Pharmaceutical
University ’N. Testemitanu’, Chisinau, Moldova
1
3
Objectives: We made a comparative analysis of low BDNF
levels in children with different grade hypoxic-ischemic
affectations of CNS
Materials and research methods: 182 children aged from
7 days to 12 months (study lot - st.lt), that suffered from
diverse grade perinatal hypoxic-ischemic injuries of the
CNS were investigated. The allotment in sub lots (sbl.) was
done according to age and clinical diagnostic. 60 healthy
children formed the control lot (control group - ctrl.gr.).
Results: The study shows that the probability of decreased
BDNF level constitutes 23.34% in children from sbl.II and
85.4% in those from sbl.III in comparison to sbl.I (3.42%).
The probability of decreased BDNF level is high in sbl.III,
especially in children with severe neurologic disorders.
Synthesis and secretion of BDNF decreases seriously in
children with severe hypoxic-ischemic injuries. Because of
low BDNF level neuroprotective and neuroregenerative
processes are inhibited.
Conclusions: It is possible that there exists an inferior limit
of BDNF level and under this limit the processes of
neuroregeneration and neuroregulation are irreversible. Or,
high BDNF level protects the brain from destruction,
sustains the survival and differentiation of neurons,
increases neural cell’s resistance to injuries induced by
ischemia. Thus, we established a strong correlation between
neurologic after effects of PHIE and concentration of BDNF
in blood. Therefore, it is the case to administrate exogenous
neurotrophic factors before the apparition of cerebral
lesional changes that is in the first minutes or hours
following the apparition of cerebral hypoxic-ischemic
injuries.
L.D. Méndez1, S. Mañas1, M.R. Duque2, L. De Vera3,
E. Pereda4, J.J. González3
1Unit of Clinical Neurophysiology, University Hospital
Nuestra Señora La Candelaria, Tenerife, 2Unit of Pediatrics,
University Hospital Nuestra Señora La Candelaria, Tenerife,
3Department of Physiology, Faculty of Medicine, University
of La Laguna, Tenerife, 4Department of Basic Physics,
University of La Laguna, Tenerife, La Laguna, Spain
Aims: To investigate age dependence of EEG functional
connectivity in children with Attention Deficit Hyperactivity
Disorder (ADHD) of mixed type, through different EEG
synchronization measures.
Methods: Signals: digitised monopolar EEGs (sampling
rate: 256Hz) from an ADHD (20-males, 5-15 years) and a
control (CONT) group (19-males, 5-15 years). Recording
electrodes: Fp1/C3/T3/O1 (reference-A2) and Fp2/C4/T4/
O2 (reference-A1). Pair-wise interdependence between
electrodes was assessed through the magnitude square
coherence MSC - in the delta, theta, alpha and beta EEG
bands- and a nonlinear index (L). Bivariate surrogate data
were used to test the significance of the interdependence
indices. Intrahemispheric (SH) - for each region/electrode
of the left and right hemisphere- and interhemispheric (DH)
connectivity, were assessed by averaging the corresponding
intrahemispheric/interhemispheric indices for each
electrode. Conditions were basal eyes-open (OE) and eyesclosed (CE). MANOVA test for repeated measures and posthot Bonferroni were used for between-group and age
comparisons.
Results: Unlike CONT children, ADHD children younger
than 10 years showed significant greater cortical
connectivity (assessed through MSC) than those older than
10 (p<0.01 for delta band, OE and DH and p<0.05 for delta
band during CE and SH+DH). ADHD children showed a
significant greater synchronization than CONT for the L
index (p<0.01 during CE and SH) but no age dependence
was observed by this index.
Conclusions: Age of ADHD children of mixed type may be
relevant in assessing EEG functional connectivity
P1211
P1212
FREQUENCY OF DEVELOPMENTAL
DYSGRAPHIA
COMPLICATIONS OF INTRATHECAL
BACLOFEN PUMP: PREVENTION AND
CURE
S.M. Golubovic1, G. Colic2
1Speech and Language Pathology, Faculty of Special
Education and Rehabilitation, University of Belgrade,
2Faculty of Special Education and Rehabilitation, Belgrade,
Serbia
Introduction: Developmental dysgraphia is a brain-based
type of learning disability that specifically impairs a child’s
ability of writing. More than 50% of children having SLI
continue on to develop dyslexia and dysgraphia. Dysgraphia
may overlap with related conditions such as dyspraxia,
attention deficit disorder and dysphasia (SLI). The aim of
this study was to determine the frequency of appearance of
dysgraphia in younger school children according to gender,
type of dysgraphia and the frequency of errors in writing.
Methodology: The sample consisted of 190 children of
first, second, third and fourth grade of primary school
"Vladislav Petkovic Dis" in Belgrade. Examination was
done during May and June 2010 using the scale for
evaluating the level of handwriting dysgraphia (Cordic &
Bojanin) Subtest XI Acadia test of developmental abilities
(Atkinson S, Johnston B & A. Lindsay) and specially
designed protocol for dysgraphia. The study was conducted
in three phases.
Research results: The total sample is comprised of 190
children, among whom most were children of fourth grade
(28%), slightly more girls (52%) than boys (48%). There
was a total incidence of dysgraphia in 18% of children, the
presence of a higher percentage of dysgraphia in boys
(12%) than girls (6%), and higher frequency of dysphasic/
dyslexic dysgraphia (12%) compared to the perceptive
dysgraphia (6%).
Conclusion: A high incidence of dysgraphia was found,
particularly in dysphasic-dyslexic dysgraphia in relation to
the graphomotor, in this examination in comparison to our
previous examinations and suggests a worrying phenomenon
of language disorders in these children.
141
Y. Awaad
KFMC, Riyadh, Saudi Arabia
Increasingly, spasticity is managed with surgically
implanted ITB pumps. ITB revision surgery unrelated to
programmable pump end-of-life is not uncommon, requiring
special attention during pre-, intra-, and post-operative
management. We aim to identify and observe complications
of ITB (Intrathecal Baclofen) pump as well as to report
avoidance and cure of complications.
Materials and methods: Through 2002-2006, we
implanted ITB pumps in 44 patients; 24 children versus 20
adults; 30 «primary-implant-patients»; 14 «revision-only
patients». We evaluated reasons for revision surgeries and
diagnostic work-up requirements.
Results: 8 out of 30 «primary-implant-patients» required
14 revisions and 7 of 11 «revision-only-patients» needed 13
procedures. 7 patients with slowly increasing baclofenresistant spasticity had either:
1) Unsuspected pump-catheter connector defects (n=4) with
a subcutaneously dislocated IT (intrathecal) catheter (n=1),
2) An X-ray-documented pump-catheter connector defect
(n=1)
3) X-ray-demonstrated fractured catheter with intrathecal
(IT) fragment (n=2) requiring laminectomy (n=1).
Injection studies revealed IT peri-catheter arachnoiditis
(n=1); managed without laminectomy, and connectorrelated dye leakage (n=3). Implant infections occurred in 4
cases (3 were pre-operated). Scintigraphy revealed occult
CSF leakage (n=1). Intrinsic pump failure (n=1).
Conclusions: ITB, although very gratifying, has a high,
technique-related complication incidence during implant
life. Meticulous technique, high clinical suspicion,
appropriate work-up, and timely surgical management can
reduce surgical ITB complications.
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P1213
P1214
AUTISM AND CELIAC DISEASE: FAILURE
TO VALIDATE THE HYPOTHESIS THAT A
LINK MIGHT EXIST
ISOSORBIDE DINITRATE (ISDN) IS A SAFE
PROVOCATIVE AGENT IN HEAD UP TILT
TEST (HUT) IN CHILDREN AND
ADOLESCENTS
R. Abolfazli, A. Mirbaghri, A. Zabihi, S. Samadzadeh
Tehran University of Medical Sciences- Amir Alam Hospital,
Tehran, Iran
Background: Autism is a heterogeneous condition and the
possible pathogenic role of several different factors has
been postulated. Previous studies reported the existence of
a linkage between autism and celiac disease (CD). The aim
of this study was to determine the association between
autism and CD by anti-gliadin (AGA), anti-endomysial
(AEA) and tissue transglutaminase (tTG) antibodies.
Materials and methods: 34 consecutive autistic children
(18 boys and 16 girls, 9.2±4.1 years old range 4-16 years)
and 34 age- and sex-matched healthy anonymous blood
donors (18 boys and 16 girls, 10.8±4.0 years old range 4-16
years) were included. None of the patients and controls had
symptoms (or positive family history) suggestive of specific
gastrointestinal diseases. AGA and AEA antibodies (IgG
and IgA) and IgA-tTG were detected by ELISA. Individuals
with positive serology were offered duodenal biopsies.
Results: IgG-AGA was found in 4 (11.8%) patients and 2
(5.9%) controls (p=0.69), while IgA-AGA was found in
none of patients and controls. All patients presented normal
values of IgG and IgA-AEA similar to control. There was
no significant relationship between levels of AGA and AEA
antibodies and the severity of autism in patients groups. The
levels of IgA-tTG in four patients (but no controls) were in
the borderline range (p=0.11) and two of them were found
to have mild villous changes with chronic inflammatory
cells. However, characteristic histological features of CD
were absent.
Conclusions: No evidence was found that children with
autism were more likely than children without autism to
have celiac disease.
M.R. Sabri1, M. Majid2, Children
Pediatrics, 2Isfahan University of Medical Sciences,
Isfahan, Iran
1
Introduction: HUT is used in diagnosing children and
adolescents with NMS. Conventional HUT protocol is timeconsuming and sensitivity of this method is low. Provocative
agents such as nitroglycerin and ISDN are commonly used
to increase diagnostic yield of HUT. Sublingual ISDN-HUT
is suitable for routine clinical practice in children with
unexpected syncope. This study evaluated the advantage
and safety of a short ISDN-HUTprotocol for diagnosing
NMS in children and adolescents.
Material and methods: We studied 172 patients (84 males)
referred with unexplained syncope. In the case group 43
patients had mean (±SD) age of 11.8±3.7 years and in
control group 129 patients had mean (±SD) age of 11.7±3.7
years. The patients in the case group were tilted to an angle
of 65 degrees for 20 minutes. If the test became negative the
patient would receive 0.1mg/kg ISDN in supine position.
After 5 minutes, the table was tilted to an angle of 65
degrees for a maximum of 20 minutes or until the test
became positive. The patients in the control group were
tilted to an angle of 65 degrees for 40 minutes (conventional
HUT protocol).
Results: Overall 36 patients (83.5%) in the case group 17
patients (39.5%) in unmedicated phase and 19 patients
(44%) in medicated phase and 80 patients (62%) in the
control group had a positive response (p=0.009). All patients
in the case group tolerated ISDN very well.
Discussion: Our finding showed that short ISDNHUTprotocol for diagnosing NMS is safe and has a higher
rate of positive response than conventional HUTprotocol.
P1215
Abstract cancelled
143
P1216
P1217
VISUALY EVOKED POTENTIALS IN
INFANTS WITH SYMPTOMATIC EPILEPSY
SEMANTIC ABILITIES IN CHILDREN WITH
READING DISABILITY
M.I. Sigatullina1, S.S. Shomansurov1, D. Sattarova2
S.M. Golubovic, T. Lukovac
Child Neurology, Tashkent Institute of Postgraduate
Medical Education, 2Neurology, Tashkent Medical Academy,
Tashkent, Uzbekistan
Speech and Language Pathology, Faculty of Special
Education and Rehabilitation, Belgrade, Serbia
1
Objective: In infants with symptomatic seizures it is very
difficult to predict accurately neurologic outcome and
evaluate the visual functions. Visual evoked potential (VEP)
is a reproducible measure of cortical function and can
predict the neurodevelopmental outcome. We performed
VEPs on 50 patients from 3 months to 3 years to investigate
the relationship between VEPs and neurodevelopmental
outcome.
Method: The 1st group consisted of 30 infants with focal
symptomatic epilepsy, the 2nd - 10 infants with generalized
symptomatic seizures, the 3rd - 10 healthy infants.
Results: The maximum delay of latency (37, 4±30 mc) of
component P100 of the VEPs and more expressed
disturbances of carrying out of impulses on visual ways
were found in 1st group. In patients from the second group
the latency delay and decrease of amplitude have been less
expressed in comparison to patients with focal epilepsy. In
32% of patients from the first group the cortical visual
impairment with disturbance of visual reactions and optic
nerve hypoplasia have been determined. Severe motor and
psychological disorders in 36% of children from the 1st and
26% in 2nd group were observed. In 3rd group all had normal
VEPs and neurodevelopment during the first 6 months of
life.
Conclusion: Delay of latency and decrease of amplitude of
the main VEPs component are very specific findings in
children with symptomatic epilepsy. VEPs demonstrate
quite good correlation with neurodevelopmental outcome in
infants with symptomatic epilepsy and are very useful in the
clinical management of these infants.
Introduction: We examined the connection between
semantic abilities (ability of recognition relations and
combining meaningful forms of speech through homonyms,
antonyms and synonyms, ability of generalization of
noticing general in specific meaning, and ability of noticing
specific in general meaning) and mastering the ability of
reading (operationalized through the reading speed, errors
in reading the number of memorized facts).
Methodology: The sample included a total of 314 children,
286 without disabilities in reading, 28 children with
disabilities in reading in lower grades of primary school. All
children were tested by the same measuring instruments
(tests).
Result: Of the total sample of children without disabilities
in reading 52.6% were boys, 47.4% girls, 2.1% of children
attended the first, second 55.8%, 7% the third, and 35.1%
the fourth grade. In relation to handedness, most were
dislateralized (49.5%), then right-handed (45.6%), and the
least left-handed (4.9%). The sample of children with
disabilities in reading (28), rather uneven at age, boys make
up 57.1% of the sample (16) and girls 42.9% (12), of which:
12 children are right-handed, and the other 16 dislateralized;
24 of them in the moment of conducting research attended
second grade, while in third grade was one child, and in
fourth three of them. There are significant differences
between children without disabilities in reading and children
with disabilities in reading compared to all three measured
semantic abilities. Children with reading disabilities show
significantly lower average scores on tests of examined
semantic abilities.
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P1218
P1219
CLINICAL FEATURES OF PAROXYSMAL
NON-EPILEPTIC EVENTS IN DIFFERENT
PEDIATRIC AGE GROUPS
CAMURATI-ENGELMANN DISEASE
SIMULATING A NEUROMUSCULAR
DISORDER
J. Delic1, D. Nikolic2, N. Dimitrijevic2, D. Bogicevic2,
B. Medjo3, A. Cvijovic1
F. Correia1, M. Cordeiro2, A. Cerqueira3, P. Carneiro4,
R. Gomes5
School of Medicine, University of Belgrade, 2Pediatric
Neurology, 3Pediatric Intensive Care Unit, University
Children’s Hospital, Belgrade, Serbia
Neurologia, Hospital Pedro Hispano, Matosinhos,
Pediatria, Centro Hospitalar Vila Nova Gaia / Espinho, Vila
Nova de Gaia, 3Pediatria, Hospital de Braga, 4Neurologia,
Centro Hospitalar Vila Nova Gaia / Espinho, Vila Nova de
Gaia, 5Pediatria, Hospital Pedro Hispano, Matosinhos,
Portugal
1
Introduction: The paroxysmal non-epileptic events (PNES)
in childhood are a group of disorders, syndromes and
phenomena that mimic true epileptic seizures. The
differential diagnosis of PNES is very broad spanning a
wide variety of physiologic, organic and behavioural
conditions. The key to diagnosis is a detailed history and
careful observation.
Objective: To assess the etiology of childhood PNES in
different age groups.
Methods: Retrospective study was conducted on 59 patients
(20 boys and 39 girls) aged 1 month to 18 years with history
of PNES. The follow-up period was 1.5 years. The etiology
of non-epileptic events was assessed and compared
afterwards according to age.
Results: The most frequent cause of PNES was syncope
(30.5%), followed by psychogenic non-epileptic seizures
(18.6%). 11.9% had apparent life-threatening events
(ALTE) as an underlying cause, 8.5% hypoglycaemia, 6.8%
some kind of intoxication and 3.4% migraine headaches,
whereas 20.3% of cases of PNES were due to other causes
(dehydration, benign paroxysmal vertigo, post-vaccine
reactions, masturbation-like movements). We noticed more
frequent occurrence of ALTE in infancy (5.57±6.29 months)
and syncope (155.16±36.13 months) and psychogenic nonepileptic seizures (168.64±52.07 months) in adolescence.
Gender was not shown to be a significant factor.
Conclusion: Our study has shown that organic disturbances
were the most frequent causes of PNES in infants and
toddlers, whereas autonomic dysfunction disorders and
psychogenic non-epileptic seizures were predominantly
noticed in older age groups.
1
2
Introduction: Camurati-Engelmann Disease or progressive
diaphyseal dysplasia is a rare autosomal dominant disease,
with a variable clinical presentation and characterized by
cortical thickening of the diaphyses of long bones. We
present a case whose clinical manifestations suggested a
neuromuscular disease.
Clinical case: We report a 10-year-old boy, with a history
of progressive waddling gait, frequent falls, difficulty in
climbing stairs, inability to run and widespread pain in the
legs. His father had a history of proximal lower limb pain
and change in gait since childhood, never investigated. The
neurological examination showed decreased proximal leg
strength with positive Gowers manoeuvre, normal tone,
absence of muscle atrophy and normal deep tendon reflexes.
The gait was broad based with large swing of the hip. In the
analytical study, there were no changes in hemogram,
sedimentation velocity, creatine kinase, renal function,
phosphocalcic metabolism and alkaline phosphatasis.
Magnetic resonance imaging of the neuraxis, electro
myography and muscle biopsy were normal. Cortical
sclerosis and thickening were found in the diaphysis of the
long bones of his arms and legs in bilateral and symmetric
ways suggestive of a progressive diaphyseal dysplasia. The
diagnosis was confirmed by demonstration of mutation in
the TGFβ1 gene. Later, the same mutation was also found
in the father. The patient started corticosteroid therapy and
showed progressive improvement of pain complaints.
Conclusion: Although the Camurati-Engelmann Disease is
well described, the authors suggest that, neurologists and
neuropediatrics should remember to X-ray the long bones
of “myopathic” children, and offer steroids when the clinical
condition warrants.
145
P1220
P1221
ROBOTIC GAIT TRAINING IN
CHILDREN WITH CEREBRAL PALSY:
STATE-OF-THE-ART
THE ASSOCIATION BETWEEN ADHD AND
IMPAIRMENT IN COGNITIVE DOMAINS
F. Molina Rueda1, M. Carratalá Tejada1,
I.M. Alguacil Diego2, A. Molero Sánchez1,
J.C. Miangolarra Page2
Department of Neurology, Dr Sardjito Hospital, Gadjah
Mada University, Yogyakarta, Indonesia
Universidad Rey Juan Carlos, Alcorcón (Madrid),
2Fisioterapia, Terapia Ocupacional, Rehabilitación y
Medicina Física, Universidad Rey Juan Carlos, Alcorcón,
Spain
1
Background: The sustained growth of interest in therapeutic
robotics in recent years is due to a significant shift away
from assistive technology for people with disabilities toward
robotic therapies, which use the technology to support and
enhance clinicians´ productivity and effectiveness as they
try to facilitate the individual’s recovery.
Purpose: The aim of this review is to summarize the current
research evidence on robotic-assisted treadmill therapy in
children with Cerebral Palsy and its effects on functional
outcomes in them.
Methods: We selected studies with all research designs
including case reports and fully published studies (not
abstract) in peer-reviewed journals. Studies showing the
effectiveness of robotic-assisted treadmill therapy in
children with Cerebral Palsy under 18 years of age.
Results: All studies, with the exception of one, consisted of
cohort studies, indicating that the studies’ level of evidence
was low. Robotic-assisted locomotion training significantly
improves the GMFM, walking speed and covered distance.
Interpretation: This systematic review is limited by the
small number of participants, heterogeneous level of
abilities of participants and low quality of the trials. Highquality randomized, controlled, clinical trials should be
completed to determine whether robotic-assisted locomotion
training programs have a beneficial effect on functional
locomotion for children with Cerebral Palsy.
Conflict of interest: This article is conducted under the
framework of hyper.
C. Thursina
Background: ADHD is one of the most common disorders
found in children. Cognitive impairment is often found in
children; however, it is often missed. Furthermore, there is
still some debate over which cognitive domains are affected
by ADHD.
Objective: To examine the general cognitive function as
well as specific cognitive domains in children with ADHD.
Subjects and method: This cross-sectional study was
conducted between August and November 2009 in the
district of Banguntapan. Children between 6-11 years of
age, who qualified based on pre-specified criteria, were
enrolled in this study. All subjects were screened with
DSM-IV, ACTRS and ACPRS, Mini Mental State
Examination Child (3MSEC) and Wechsler Intelligence
Scale for Children (WISC-III).
Results: From 2,021 children registered in 6 elementary
schools in Banguntapan, 55 were reported as ADHD cases
(2.87%), in which 35 were entered in analysis. There were
no differences between case and control group regarding
demographic variables. In 3MSEC, children with ADHD
appeared to have more difficulties in attention, recall and
identity (p=0.001, p=0.04 and p=0.001, respectively).
Moreover, children with ADHD had significantly lower IQ
compared with normal children (p=0.017), more specifically
in performance IQ (p=0.044). In the IQ subcomponents,
ADHD children had lower score in calculation and
information (p=0.019 and p=0.016, respectively).
Conclusion: ADHD children may have lower cognitive
function, especially in attention, recall and language. ADHD
children also appeared to have lower IQ score, especially in
performance IQ.
146
P1222
P1223
GUILLAIN-BARRÉ SYNDROME IN
INFANCY: A STUDY of 9 CHILDREN
FIRST EXPERIENCE OF USING
BOTULINUM TOXIN IN TREATMENT OF
SPASTIC CEREBRAL PALSY IN the
REPUBLIC OF MOLDOVA
F. Jardak1,2, E. Ellouze1,2, I. Hsairi1,2, I. Ayadi1,2,
F. Kamoun1,2, C. Triki1,2
Department of Child Neurology, Hedi Chaker Hospital,
Research Unit of Pediatrics Neurology, 01/UR/08-05, Sfax,
Tunisia
1
2
Object of the study: Guillain-Barré syndrome (GBS) is the
most frequent cause of flaccid paralysis in children since the
polio vaccine was used routinely. The incidence is far less
important than in adults. This study describes clinical
features and outcomes after treatment of GBS in 9 children.
Material and methods: All patients had a clinical exam
and electromyography. Cerebrospinal fluid examination
help establish the diagnosis in 7 cases. In one case medullar
MRI was done for sphincter troubles.
Results: There were 4 girls and 5 boys aged 18 months to
13 years. Antecedent infection within 3 to 4 weeks prior to
symptom onset was found in 7 cases and influenza
vaccination in 1 case. According to the clinical and
electrophysiological features our patients were classified to:
Acute inflammatory demyelinating polyradiculoneuropathy
(AIDP) (7/9), and acute motor axonal neuropathy (2/9).
According to the clinic progression, treatment associated
general supportive treatment and physical rehabilitation, 5
patients had intravenous immunoglobulin and artificial
ventilation was necessary in 2 patients with AMAN form;
one of them died. Progressive recovery over months was
seen in all patients but was slower in the AMAN form. Two
patients still have residual functional deficit and one child
had a recurrent GBS.
Conclusion: As classically described, an infection was
incriminated in most cases. Although the evolution is
globally benign in GBS, the prognosis can be poor, even
leading to death in some cases. Recurrent GBS is rarely
described in children. The treatment has to be
multidisciplinary to prevent residual effects.
T. Cucu, A. Nacu, A. Siric
Outpatient Department, Child Rehabilitation Center,
Chisinau, Moldova
Cerebral Palsy (CP) is the most common motor disorder in
children. Hypertonicity is a leading cause of disability for
children with CP. There are numerous treatment options for
the hypertonicity in CP. Our aim was to determine the
efficacy of botulinum toxin as a part of comprehensive
rehabilitation treatment in spastic cerebral palsy. Botulinum
toxin type A (Dysport) is used as treatment of spasticity in
the Child Rehabilitation Center since 2009. Two groups of
patients with spastic form of cerebral palsy were included
in the study. The first group of 38 patients received treatment
with botulinum toxin type-A and rehabilitation treatment
(physiotherapy, occupational therapy, hydrotherapy,
dynamic proprioceptive correction). The control group
included 30 children who received rehabilitation treatment,
without treatment with botulinum toxin. The grade of
spasticity was assessed by Modified Ashworth Scale for
spasticity. The grade of motor function lesion was evaluated
by GMFCS and GMFM-66. The results of this study
confirmed the efficacy of combined use of Dysport and
rehabilitation treatment which were conducted after the
drug injection. The use of dynamic proprioceptive correction
method led to the consolidation of corrected motor
stereotype.
147
P1224
P1225
QUALITY OF LIFE OF CHILDREN WITH
POST-TRAUMATIC EPILEPSY RECEIVING
VALPROATES AND LEVETIRACETAM
TREATMENT with BOTULINUM TOXIN
TYPE-A INJECTION IN CHILDREN WITH
CEREBRAL PALSY
A. Prokhorova
N. Kuzmina1,2, Z. Rafikova3, I. Yuldashev1, L. Sakhieva4
Tashkent Medical Academy, Tashkent, Uzbekistan
Urban Medical Children Diagnostic Centre, 2’Safo Tibbiyot’
Clinic, 3Urban Children Clinic Nr1, 4’Evromed’ General
Clinic, Tashkent, Uzbekistan
According to the recommendations of the ILAE experts,
posttraumatic epilepsy (PTE) is one of the most common
etiopathogenetic types of locally caused symptomatic
epilepsy. The major requirement to a therapy is to avoid
negative effects on quality of patients’ life. The objective of
our research was to examine the quality of life of children
with PTE receiving valproate (VP) and levetiracetam
(LEV).
Material and methods: 33 patients aging from 11 to 14
years diagnosed with PTE. The quality of patients’ life was
studied using questionnaire QOLIE-AD-48, version 1. All
patients included in the research received the AED monotherapy. Group I was composed by 18 patients receiving VP,
group II consisted of 18 patients receiving LEV.
Results: We have analyzed the effects of VP and LEV on
cognition and emotional status, being the major indicators
of life quality. The mean score of cognitive functions of the
group I children was 33.8±0.57 and in the group II children,
it was 41.2±0.65. It is quite probable that the Keppra
positive effect on cognitive functions of PTE children is
caused by nootropic action of the drug. The mean score of
social activity in group I made 32.5±0.58, while in group II,
it was 43.6±0.42 (p<0.001).
Conclusion: The comparative analysis of using VP and
LEV in treatment of PTE children has shown that despite
high anti-epileptic activity of the both drugs, LEV
administration contributes to achievement of higher
indicators of patients’ life quality.
1
Introduction: Cerebral Palsy (CP) is the most common
cause of spastic movement disorders in children. Among
pharmacological treatments of spasticity Botulinum Toxin
type A (BTX-A) has acquired an important role in treatment
of CP. Spastic depligia is the most common form of the
spastic forms of CP.
Aim: To investigate functional motor outcome in two
groups of children with CP.
Patients and methods: 39 children (ranged 2-12 years)
with CP were divided in two groups: the first group (19
children) received BTX-A and physiotherapy, occupational,
speech therapy, orthoses and the second group (18 children)
received only physiotherapy, occupational, speech therapy,
orthoses. BTX-A injections to lower limbs were performed
in the following muscles: m. adductor magnus, m. adductor
longus, m. adductor brevis, m. gracilis, m. semimem
branosus, m. semitendinosus, m. gastrocnemius, m.soleus.
We use BTX-A in doses 15-30U/kg divided bilaterally to
necessary amount of muscle targets. To compare these
groups we used modified Ashworth scale, Gross Motor
Function Classification System (GMFCS) and EMG.
Measurements were performed before, after 4 and 16 weeks
of intramuscular injection of BTA.
Results: All children with CP from the first group showed
an improvement of dynamic gait pattern after injection of
BTX-A compare to the second group. Occurrence and
severity of adverse events were very rare. BTX-A allowed
stretching and improved motion with benefits lasting up to
6 months.
Conclusion: Intramuscular injection of BTX-A is a safe
and effective treatment for reducing disability in children
with CP.
148
P1226
P1227
DE NOVO 7Q DELETION IN A FAMILY
WITH 16P DUPLICATION, AUTISM AND
DYSLEXIA
TWO DIFFERENT CASES OF
HEMIMEGALENCEPHALY WITH
HYPOMELANOSIS OF ITO AND WEST
SYNDROME
G.J. Braathen1,2, A.K. Eek1, R. Stornes3, K.O. Clausen1
1Department of Laboratory Medicine, Section of Medical
Genetics, Telemark Hospital, Skien, 2Head and Neck
Research Group, Research Centre, Akershus University
Hospital, Lørenskog, 3Department of Pediatrics, Telemark
Hospital, Skien, Norway
Introduction: Copy number variations (CNV) in several
chromosomal regions have been associated with increased
risk of autism spectrum disorders (ASD). Chromosomal
regions implicated in studies of ASD and associated
phenotypes such as schizophrenia, mental retardation and
developmental delay include 1p, 1q, 3p, 5q, 7q, 10q, 15q,
16p, 17p, 20p, 22q and Xq.
Methods: We describe a child with de novo 7q deletion and
maternal 16p duplication, the genetic analyses performed in
the family and the familial phenotypes.
Results: The chromosomal analyses were normal while
array Comparative Genomic Hybridization (aCGH)
identified the 7q deletion and the 16p duplication.
Conclusions: ACGH makes it easier to assess different
clinical aspects of neurodevelopmental disorders such as
autism and may have clinical and pedagogical impact.
M.I. Sigatullina, S.S. Shamansurov, N.A. Mirsaidova
Child Neurology, Tashkent Institute of Postgraduate Medical
Education, Tashkent, Uzbekistan
Objective: We investigated the clinical features of
Hemimegalencephaly in two different patients: one patient
with Hypomelanosis of Ito and another one with West
syndrome.
Case report: The first patient, a 3-year-old girl was the first
born child of a third degree consanguineous Muslim couple.
Hypopigmented lesion over the right part of the face has
been noticed since the birth. She developed focal seizures
with tonic posturing of the right upper limb on the first
month of life. She also had a total left sided hemihypertrophy.
Neuropsychological examination revealed mental
retardation. A clinical diagnosis of Hypomelanosis of Ito
was made. MRI of the brain revealed generalized
enlargement of left cerebral hemisphere with cortical
thickening consistent with hemimegalencephaly. The other
patient a 16-month-old girl was referred with complaints of
polymorphic seizures, mental and motor retardation,
weakness of the right head and leg. Intractable epilepsy
started during the first months of life and included a
combination of spasms, focal and myoclonic/atonic
seizures, along with psychomotor retardation and
dysmorphic features. The EEG was characterized by
asymmetrical hypsarrhythmia in the left hemisphere.
Neurological examination revealed right spastic
hemiparesis. MRI of the brain revealed left hemimegal
encephaly with pachygyria. A clinical diagnosis of
symptomatic West syndrome was made and presurgical
evaluation for functional left hemispherectomy was
performed.
Conclusions: Common clinical manifestations of
Hemimegalencephaly may occur as an isolated brain
malformation or in association with neurocutaneous
syndromes, as in the present case and can include mental
retardation, seizures, language disabilities, and motor
system dysfunction.
P1228
P1229
RECURRENT HEADACHES IN CHILDREN
AND ADOLESCENTS MAY BE CAUSED
BY CEREBRAL TOXOPLASMOSIS (T.)
A DOWNWARD SPIRAL
A. Fuglewicz1, J. Prandota1, A. Gryglas1,
B. Ujma-Czapska2, L. Szenborn3, J. Mierzwa4
Social Pediatrics, University School of Medicine, Pediatric
Neurology, Marciniak Specialistic Hospital, 3Pediatrics and
Infectious Diseases, 4Neurology and Neurosurgery,
University School of Medicine, Wroclaw, Poland
1
2
Introduction: T. gondii usually infects about one third of
the world’s population and it is believed that in
immunocompetent hosts the infection is usually
asymptomatic. Recently, Koseoglu et al. showed that in a
group of 104 adult patients with migraine, 46 subjects were
seropositive for anti-T. gondii IgG antibodies compared
with controls.
Aim of the study: The purpose of the study was to estimate
T. infection frequency in the population of children
hospitalized with symptoms and/or signs of the central
nervous system abnormalities, and to establish if it was an
acute or chronic infection.
Methods: The study was performed in 93 children aged
1-17 years admitted consecutively to the hospital from
November 2009 to May 2010. Blood samples were taken on
admission and studied for the presence of specific anti-T.
gondii IgM, IgG antibodies and index of IgG avidity in
serum using enzyme immunoassay Platelia Toxo IgM, IgG,
and IgG avidity.
Results: 10 children (5 boys; 8-17 yrs old, mean age 14.2
yrs) had high anti-Toxoplasma gondii IgG antibody blood
levels (range: 40.5-240 UI/ml, mean 142,19 UI/ml; the
positive value for IgG was ≥9 UI/ml). The avidity Index
(AI) ranged from 0.67 to 0.859 (scale: ≥0.5 high AI). The
headaches affected frontal area (10 children), occipital (4),
and parietal areas (5). The most frequent comorbidities
included epilepsy, upper respiratory tract infection, allergy,
abnormalities of behaviour, attention disturbances.
Conclusion: 10.7% of 93 studied children had specific
anti-T. gondii IgG antibodies and high index of avidity
suggesting chronic infection.
149
P.H. McNamara1, M. Farrell2, J. Redmond1
1Neurology, St. James’s Hospital, 2Neuropathology,
Beaumont Hospital, Dublin, Ireland
We present this 23-year-old lady with a history of learning
difficulties from late childhood, hearing impairment from
the age of 14, ataxia from the age of 16, seizures from the
age of 18 and dysphagia at age 22. She required bilateral
hearing aids. Birth history and family history were
unremarkable. Parents were non consanguineous. Positive
Evolving examination findings showed a vertical gaze
palsy, dysarthria and ataxia of both upper and lower limbs
and also gait. Investigations showed positive tTG and antiendomysial antibodies. CSF exam was normal. MRI brain
showed cerebellar atrophy. Muscle biopsy was normal. EEG
showed bilateral dysfunction. During a hospital admission
she had a generalized seizure and was noted to be cyanosed
and pulseless. CPR was commenced with spontaneous
return of circulation. Telemetry later showed a short run of
SVT with no symptoms. ECHO was normal. A reveal
device was inserted and showed normal sinus rhythm.
Unfortunately in June 2010 she died aged 23. Post-mortem
examination showed splenomegaly and cerebral neuronal
cytomegaly with vacuolar change and widespread Tau
positive neurofibrillary tangles consistent with Niemann
Pick type C disease. Genetic analysis revealed that she was
a compound heterozygote for two NPC1 mutations.
150
P1230
P1235
EPILEPSY IN CHILDREN WITH
CEREBRAL PALSY
CONTEMPORARY ASPECT OF
CRANIOFACIAL MALFORMATION IN
NEWBORNS AND INFANTS IN THE
REPUBLIC OF MOLDOVA
N. Kitchener1, H. El-Khayat2, S.S. Aziz3, N. Nagy3
1Neurology, Mataria Teaching Hospital, General
Organization for Teaching Hospitals and Institutes,
2Pediatrics, 3Psychology, Ain Shams University, Cairo, Egypt
P1231
SEIZURES IN the NEONATAL INTENSIVE
CARE UNIT
B. Medjo1, D. Nikolic2, M. Atanaskovic-Markovic3,
G. Cuturilo4, S. Rsovac1, N. Vunjak1
Pediatric and Neonatal Intensive Care Unit, 2Department of
Neurology, 3Deaprtment of Pulmonology and Allergology,
4Department of Genetics, University Children’s Hospital,
Belgrade, Serbia
1
A. Railean1, G. Railean2, S. Hadjiu1, I. Ilciuc2
Neuropediatrics, State University of Medicine and
Pharmacology “NicolaeTestemitanu”, 2Neuropediatrics,
IMSP Institute of Neurology and Neurosurgery RM,
Chisinau, Moldova
1
P1236
NEUROLOGICAL FEATURES IN
ONTOGENESIS OF POSTERITY OF HIGH
RISK AND FALLING ILL A
SCHIZOPHRENIA
M.A. Kalinina1, Y.B. Kalyn2
Department of Psychopathology of Early Childhood,
Department of Research of Alzheimer Disease, MHRC
RAMS, Moscow, Russia
1
P1232
2
DANDY WALKER SYNDROME
ASSOCIATED WITH DOWN SYNDROME
M. Rakusa1, V. Savanovic1, T. Seruga2, N. Kokalj Vokac3,
A. Zagorac3, H. Bozic1
Department of Neurology, 2Department of Radiology,
3Laboratory of Medical Genetics, University Medical Centre
Maribor, Maribor, Slovenia
1
P1237
The WAY OF FORECASTING
CONVULSIVE SYNDROME in CHILDREN
WITH CONGENITAL HEART DISEASES
M.A. Nurmukhamedova
P1233
INDICATORS OF NEUROPSYCHOLOGICAL DEVELOPMENT OF A
NEWBORN WHO TRANSFERRED HEAVY
PERINATAL PATHOLOGY
F. Samadov, U. Abdukadirov, S. Kabilov
Andijan State Medical Institute, Andijan, Uzbekistan
P1238
PERINATAL FACTORS AND CEREBRAL
NEURONAL PROCESSES IN
ADOLESCENTS WITH VEGETATIVE
DISORDERS
G. Railean
P1234
RISK FACTORS AND ETIOLOGIC PROFILE
IN ROMANIAN CHILDREN WITH
CEREBRAL PALSY (CP)
E. Rosulescu1,2, C.V. Albu3,4, M. Zavaleanu1,5
Department of Kinetotherapy, University of Craiova,
2Children Neurorehabilitation Center, DGASPC, 3Neurology
Department, University of Medicine, 4Neurology Clinic,
Hospital No.4, 5Sama Medical Center, Craiova, Romania
1
Neurology, Tashkent Pediatric Medical Institute, Tashkent,
Uzbekistan
Neuropediatrics, IMSP Institute of Neurology and
Neurosurgery RM, Chisinau, Moldova
P1239
CNS INFECTIONS AS A CAUSE FOR
EPILEPSY
O. Jotovska
Institute for Mental Health of Children and Youth, Skopje,
FYR Macedonia
P1240
P1243
THE INTERDISCIPLINARY COOPERATION
BETWEEN SPECIALISTS FOR THE EARLY
DIAGNOSIS OF NEUROFIBROMATOSIS
THE CASE FOR A GENDERDIFFERENTIAL-APPROACH TO THE
EVALUATIONS/INTERVENTIONS IN
UNCOMMON CHILDHOODPAROXYSMAL-EVENTS
B. Gjoni1, D. Naco2, N. Burda3, E. Muzhaqi4
Dermatology, 2Neurology, 3Ophthamology, 4Radiology,
Outpatients Unit Nr 2, Tirana, Albania
1
151
E.U. Onyekwelu
Paedaitrics, Royal Victoria Teaching Hospital, Banjul, The
Gambia
P1241
STANDARDS OF NEUROTROPHIC
THERAPY AT PREVENTIVE
MAINTENANCE AND TREATMENT OF
DISTURBANCES OF A CEREBRAL
CIRCULATION AFTER CARDIOSURGERY
R. Ibadov1, M. Nurmukhamedova2, A. Arifjanov1,
N. Strijkov1
The Specialized Center of Surgery V.Vakhidov, 2Tashkent
Pediatrical Medical University, Tashkent, Uzbekistan
1
P1244
SERUM MELATONIN LEVEL IN CHILDREN
WITH SIMPLE, COMPLEX FEBRILE
SEIZURES AND EPILEPSY
A. Mahyar1, P. Ayazi1, N. Gholami1,
M.M. Daneshi-Khohan1, N. Mohammadi1,
M.H. Ahmadi1, A.A. Sahmani1, S. Mahyar2
1Qazvin University of Medical Sciences, Qazvin, 2Tehran
University of Medical Sciences, Tehran, Iran
P1242
A STUDY OF CHILDHOOD PRIMARY CNS
TUMORS IN TUNISIA: SAMPLE
DESCRIPTION OF 10 CASES
I. Abid1, E. Ellouz2, I. Hsairi2, F. Jardak2,
H. Ben Othman2, F. Kammoun2, C. Triki2
1Department of Child Neurology- Hedi Chaker Hospital
Sfax, 2Reasearch Unit of Neuropediatrics (01/UR/05-08),
Department of Child Neurology- Hedi Chaker Hospital,
Sfax, Tunisia

Child and developmental neurology

Transcription

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