Full Article – PDF

CAS CLINIQUE / CASE REPORT
A PATIENT WITH HEADACHE, RIGHT UPPER EXTREMITY AND
RIGHT HEMITHORAX HYPOTHERMALGESIA
http://www.lebanesemedicaljournal.org/articles/59-2/case2.pdf
Rima EL-HERTE1, Samir ATWEH2
El-Herte R, Atweh S. A patient with headache, right upper
extremity and right hemithorax hypothermalgesia. J Med Liban
2011 ; 59 (2) : 109-111.
ABSTRACT : We present a case of partial Wallenberg
syndrome also called partial lateral medullary syndrome, a hemorrhagic or ischemic stroke of the area
fed by the posterior inferior cerebellar artery and
the clinical manifestation depends on the extension of
the lesion : dorsal-ventral, medial-lateral and rostrocaudal. Five types have been described. Our patient
had headache, hoarseness, right upper extremity, right
hemithorax and right upper gluteal hypothermalgesia
implicating the involvement of the cervical, the thoracic and part of lumbar fibers of the left lateral spinothalamic tract and the ambiguous nucleus ; an entity
not described before. The imaging done to our patient
disclosed the dissection of the left vertebral artery. He
was treated with anticoagulation with gradual improvement in his symptoms.
CASE PRESENTATION
A 55-year-old male patient with hypertension and dyslipidemia was admitted to the medical ward because of right
upper extremity hypothermalgesia noted five days prior to
presentation. The patient discovered this deficiency when
he was trying to get a bottle of cold water and noticed it
was not cold in his right hand but in his left hand. Back in
the history he notes hoarseness plus a severe continuous
aching occipital headache since five days that disturbed
his sleeping. He denies any head trauma, visual disturbances or diplopia, dysphasia, dysphagia, facial or body
dysesthesia or weakness, nausea, vomiting, dizziness,
vertigo, imbalance, or alteration of level of consciousness.
He is on indapamide 1.5 mg once daily, simvastatin 20 mg
once daily and aspirin 100 mg daily. He had bilateral
inguinal herniorrhaphy five years ago; his family history
is irrelevant; he does not smoke neither drink alcohol. His
vital signs disclosed an elevated systolic blood pressure of
155 mmHg, regular heart beats of 86 beats per minute and
a normal temperature and respiratory rate. The physical
exam showed a symmetric face, no carotid bruits; normal
Departments of Internal Medicine and Neurology, American
University of Beirut Medical Center.
Corresponding author : Rima El-Herte, MD. Department of
Internal Medicine. AUB-Medical Center. Beirut. Lebanon.
e-mail: herterima@hotmail.com
1
El-Herte R, Atweh S. Céphalée et hypothermalgésie du membre
supérieur droit et de l’hémithorax droit. J Med Liban 2011 ;
59 (2) : 109-111.
RÉSUMÉ : On présente un cas de syndrome partiel
de la fossette latérale du bulbe, une atteinte hémorragique ou ischémique du territoire vascularisé par l’artère cérébelleuse postéro-inférieure dite syndrome de
Wallenberg. La symptomatologie dépend de l’extension
de la lésion : dorso-ventrale, médio-latérale et rostrocaudale. Cinq types ont été décrits. Notre patient avait
une céphalée, une dysphonie et une diminution de la
sensation à la température et à la douleur limitée au
membre supérieur droit, à l’hémithorax droit et à la
partie glutéale droite supérieure impliquant l’atteinte du
noyau ambigu et des fibres cervicales, thoraciques et une
partie des fibres lombaires du faisceau spino-thalamique
latéral gauche. Cette entité n’a pas été décrite auparavant. Les imageries réalisées montrent une dissection de
l’artère vertébrale gauche chez notre patient dont les
symptômes se sont améliorés avec l’anticoagulation.
heart beats, good bilateral breath sound, normal exam of
the abdomen.
His neurological exam was as follows: normal cranial
nerves, pupils were reactive equal to light and accommodation, the corneal reflex was present bilaterally, no
2
FIGURE 1. T2 weighted MRI showing the hypersignal in the
lateral medulla at left (long arrow) and abolition of the flow
void in left vertebral artery (short arrow).
Lebanese Medical Journal 2011 • Volume 59 (2) 109
nystagmus, normal oral cavity with preserved gag reflex,
uvular and palatal symmetry. The tongue moved well and
there was no facial weakness. The motor power was 5/5 in
all muscle compartments. Gait and cerebellar signs (rapid
alternating movement, finger-to-nose, Romberg test, and
tandem gait) were normal. The reflexes were preserved
2/4. However, the abnormal was the sensory exam: sensation to pain and heat was absent in the right upper extremity, right hemithorax and extending to the upper outer part
of the right gluteal area. Blood metabolic profile was normal and his glucose level was normal.
Brain MRI was done and showed hypersignal band in
the lateral medulla consistent with ischemia with abolition
of the flow void signal in the left vertebral artery as seen
in figures 1, 2, 3. Subsequent cerebral angiography
showed dissection of the left vertebral artery (Fig. 4).
DISCUSSION
We presented a case of partial Wallenberg syndrome.
Wallenberg syndrome is the infarction (ischemic or hemorrhagic) of the lateral medulla and the clinical manifestation
depends on the three-dimension extension of the lesion:
dorsal-ventral, medial-lateral and rostrocaudal extension
(Fig. 5). The syndrome includes vertigo, hoarseness and
dysphagia, Horner’s syndrome and cerebellar ataxia on the
same side of the lesion, and hypothermalgesia involves the
ipsilateral face and the opposite trunk and limbs (crossed
sensory symptoms).
The contralateral deficits in pain and temperature sensation from the body are due to involvement of the lateral
spinothalamic tract; the ipsilateral loss of pain and temperature sensation from face are due to involvement of spinal
FIGURE 3. Hypersignal in left lateral medulla (arrow).
FIGURE 4. Angiography showing dissection of the left
vertebral artery (large white arrow) showing filling defect.
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
FIGURE 2. Abolition of the flow void in the left vertebral artery
(white circle).
110 Lebanese Medical Journal 2011 • Volume 59 (2)
FIGURE 5. The somatotopic distribution
of the spinothalamic and trigeminothalamic tracts
in the medulla (with the permission of the authors [1]).
R. EL-HERTE, S. ATWEH – A new partial Wallenberg syndrome
trigeminal nucleus; dysphagia, hoarseness, diminished
gag reflex are caused by involvement of the ambiguous
nucleus ; the involvement of the vestibular system causes
vertigo, diplopia, nystagmus, vomiting; the involvement
of the central tegmental tract causes palatal myoclonus
and the involvement of the inferior cerebellar peduncles
causes ipsilateral cerebellar signs including ataxia. Five
different subtypes of Wallenberg syndrome were described
in the literature by Zhang et al. [1] (Fig. 6, 7).
)
• TYPE I: Far dorsal lateral medullary infarction (
causing ipsilateral face hypothermalgesia and contralateral
body hypothermalgesia.
),
• TYPE II: Enlarged dorsal lateral infarction (
including the ventral trigeminothalamic tract causing ipsilateral and contralateral face hypothermalgesia and contralateral hypothermalgesia.
)
• TYPE III: Midlateral medullary infarction (
causing contralateral face and body hypothermalgesia.
) causing hypo• TYPE IV: Far lateral infarction (
thermalgesia in the ipsilateral face and the contralateral
lower trunk and leg.
)
• TYPE V: Restricted mediolateral infarction (
causing hypoalgesia only in the contralateral face, arm and
upper trunk, without involvement of the ipsilateral face.
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
Type I
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
Type II
FIGURE 6. Subtypes I & II of Wallenberg Syndrome (reproduced with authorization from the authors [1]).
R. EL-HERTE, S. ATWEH – A new partial Wallenberg syndrome
Lebanese Medical Journal 2011 • Volume 59 (2) 111
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
Type III
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
Type IV
Pyramidal tract
Inferior olivary nucleus
Lateral spinothalamic tract
and trigeminothalamic tract
Ambiguous nucleus
Reticular formation
Spinal trigeminal tract
and its nucleus
Solitary nucleus
Vestibular nucleus
Inferior cerebellar peduncle
Type V
FIGURE 7. Subtypes III, IV & V of Wallenberg Syndrome (reproduced with authorization from the authors [1]).
112 Lebanese Medical Journal 2011 • Volume 59 (2)
R. EL-HERTE, S. ATWEH – A new partial Wallenberg syndrome
Our patient had only hoarseness and sensory symptoms
in the right upper extremity, right hemithorax extending
to the right upper thigh area with the corresponding anatomical deficit in the left lateral medulla that should correspond to the left ambiguous nucleus and the fibers of the
cervical, thoracic and lumbar area of the left lateral spinothalamic as shown by the MRI.
Of the cross syndromes described in the literature [2],
none of the cross syndromes would fit to this patient and
the anatomical defect and the clinical manifestation corresponds to a partial Wallenberg syndrome. The vessels to
the brainstem come from the vertebrobasilar system: the
posterior inferior cerebellar artery, the anterior inferior
cerebellar artery, the superior cerebellar artery, the posterior cerebral artery, and the pontine artery. Each of these
vessels sends small branches (a few or many) into the
underlying brain stem structures along its course. Other
vessels penetrate the brain stem from the basilar artery.
Small medullary and spinal branches of the vertebral
artery make up a third group of vessels. The vessels that
are responsible for causing the Wallenberg syndrome are
the branches of the vertebral artery or, most commonly,
the posterior inferior cerebellar artery [3].
The correlation between radiologic, vascular and clinical findings has been discussed in various articles.
Ross et al. [4] described four patients in 1985 with
Wallenberg syndrome with a correlation to MRI. Early
MRI would show the lesions and allow for intervention if
needed. In our patient immediate MRI was done and
showed the abolition of the flow void and the angiography
showed dissection of the left vertebral artery and treatment was initiated to save the brainstem from further
damage.
Kim et al. [5] wrote about the correlation between the
vascular lesions and the MRI of 34 patients with different
patterns of Wallenberg syndrome. All their patients underwent angiography and their findings suggest that the larger infarct are associated with multiple vessel involvement,
dissection, poor collaterals and the smaller infarcts are
associated with single vessel, good collaterals and long
standing atherothrombotic or embolic conditions. Also the
speed of development of the vascular lesion determines
the eventual size of the infarct and consequent clinical
syndromes.
Kim [6] described in his article the symptoms-radiological correlation in 130 patients with acute lateral
medullary syndrome where he noted that the most sensory symptoms-signs were the most frequent manifestation (96%) and that the limb/body involvement without
trigeminal involvement (isolated limb/body pattern) in
21%. Headache occurred in 52%, dull, aching, throbbing
and paroxysmal, started with or before the onset of other
symptoms-signs and subsided in several days or weeks.
They occurred most often in the ipsilateral occipital area,
followed by the frontal region. According to the author, in
patients with caudal infarcts, there are less frequent presence of dysphasia and dysarthria; less bilateral trigeminal
sensory pattern, and more frequent isolated limb/body
sensory pattern and sensory gradient worse in the leg than
those with rostral lesions attributed to the lateral-superficial configuration of the infarct. The most lateral infarct
produced lesion-isolated limb/body and sensory gradient
worse in the leg due to anatomical characteristics of the
medulla: the spinothalamic fibers from the upper extremities and torso run medially deeper whereas those from the
lower extremities run laterally more superficially. Angiography showed that infarction and dissection are the most
common causes; dissection was seen more often in patients with caudal than rostral lesions.
Our patient had minimal symptoms related to the
involvement of the left lateral cervical and thoracic spinothalamic tract conducting pain and temperature sensation
from the right upper extremity, right hemithorax and the
upper outer part of the right gluteal area with a coherent
MRI of the brain stem. The sparing of the other component
of the medulla excluded the occurrence of the other items
of the Wallenberg syndrome. These may be explained by
the dissecting left vertebral artery, a rapid occurrence, with
subsequent thrombus that showered small emboli to the
brain stem causing these symptoms.
Our patient was started on oral anticoagulation and his
condition remained stable and he was discharged home.
On follow-up at seven months, he remained anticoagulated with hypothermalgesia at the upper-external gluteal
area only.
R. EL-HERTE, S. ATWEH – A new partial Wallenberg syndrome
Lebanese Medical Journal 2011 • Volume 59 (2) 113
REFERENCES
1. Zhang SQ, Liu MY, Wan B, Zheng HM. Contralateral
body half hypalgesia in a patient with lateral medullary
infarction: Atypical Wallenberg Syndrome. Eur Neurol
2008 ; 59 : 211-15.
2. Marx JJ, Thömke F. Classical crossed brain stem syndromes : myth or reality ? J Neurol 2009 ; 256 : 898-903.
3. Waxman SG. The brain stem and cerebellum. In : Waxman
SG, editor. Clinical Neuroanatomy, 26th edition, Chapter 7,
McGraw-Hill, 2009.
4. Ross MA, Biller J, Adams HP Jr, Dunn V. Magnetic resonance imaging in Wallenberg’s lateral medullary syndrome. Stroke 1986 ; 17 : 542-5.
5. Kim JS, Lee JH, Choi CG. Patterns of lateral medullary
infarction : Vascular lesion – Magnetic resonance imaging correlation of 34 cases. Stroke 1998 ; 29 : 645-52.
6. Kim JS. Pure lateral medullary infarction : clinicalradiological correlation of 130 acute, consecutive patients. Brain 2003 ; 126 : 1864-72.