Tarvinder Dhanjal
University Department of
Medicine
*Department of Radiology
Western Infirmary
Abstract
Background - We report a case of
a 61 year old man with midbrain infarction causing Claude’s syndrome
attributable to stenosis of the posterior cerebral artery.
Case Description – The patient
presented with a pupil-sparing left third nerve palsy and contralateral ataxia.
A background history of treated hypertension and cigarette smoking was obtained.
Magnetic resonance imaging revealed an area of infarction involving the left
medial midbrain. Magnetic resonance angiography revealed significant stenosis of
the left posterior cerebral artery. Antiplatelet therapy was instituted and the
patient made a satisfactory recovery.
Conclusions – This is the
first reported case of Claude’s syndrome arising in association with stenosis
of the posterior cerebral artery. Intracranial large vessel disease should be
considered as a potential aetiologic factor in patients with similar midbrain
ischaemia.
Keywords:
Midbrain infarction, Posterior cerebral artery stenosis
Case Report
A 61 year-old man was admitted
to our hospital because of ataxia and diplopia. He gave a 24 hour history of
extreme gait ataxia and diplopia which was maximal on horizontal gaze to the
right. A past history of type 2 diabetes, essential hypertension and chronic
obstructive pulmonary disease was obtained.
Examination revealed a palsy of
the left oculomotor nerve with sparing of the pupil. The gait was ataxic and
dysdiadochokinesis was present on examination of the right arm. Neurological
examination was otherwise normal. On cardiovascular examination the patient was
in sinus rhythm with blood pressure of 176 / 89 mmHg. No clinical evidence of
valvular heart disease or cardiac failure was apparent.
Magnetic
resonance imaging of brain was performed…(figure
1 and figure 2). A trans-thoracic echocardiograph
revealed left ventricular hypertrophy and mild left ventricular systolic
dysfunction but no obvious cardioembolic source.
Antiplatelet therapy with
aspirin was initiated and further secondary preventative treatment with
antihypertensive therapy was commenced after an interval of seven days. The
patient remains in a rehabilitation facility one month after ictus. No new
neurological symptoms attributable to the posterior cerebral artery stenosis
have occurred.
Discussion
There is a clear correlation
between ischaemia in the ventro-medial midbrain and the clinical presentation of
Claude’s syndrome in this case. Claude’s syndrome has been the subject of
relatively few case reports, and there is some disagreement over the precise
localisation of the culprit lesion.
In 1912, the French psychiatrist
and neurologist Henri Claude (1869-1945) described a house painter who developed
a right oculomotor nerve palsy with contralateral asynergy and extreme gait
ataxia.1 The pathological examination demonstrated a paramedian
mesencephalic infarction on the right involving a portion of the crossing of the
superior cerebellar peduncles and the medial half of the red nucleus. The medial
longitudinal fasciculus was also involved however more rostrally, the oculomotor
fibres and the entire red nucleus were infarcted.
Disagreement arises over the
involvement of the red nucleus. Coppola2 and Kremer3 both
describe the syndrome with Claude’s original red nucleus involvement. Indeed,
major neuro-opthalmology textbooks have attributed Claude’s syndrome to a
lesion of the red nucleus.4 However, a recent review of six patients
by Seo et al. demonstrated well-localised lesions on MRI in the midbrain, below
the level of the red nucleus with only minimal involvement in one case.5
There conclusion stated that the red nucleus contributes little to the syndrome
with the major pathology lying within the superior cerebellar peduncle, caudal
to the red nucleus.
The magnetic resonance images in
our case do not demonstrate involvement of the red nucleus and thus support the
theory that red nucleus involvement in not mandatory for the clinical
manifestations of Claude’s syndrome. It should be noted however that magnetic
resonance imaging abnormalities may not correlate completely with the results of
detailed neuropathological examination.
Our patient is unique as this is
the first description of a case of Claude’s syndrome as a consequence of
confirmed posterior cerebral artery (PCA) stenosis. PCA territory infarction is
uncommon, accounting for only 5-10% of entries in most stroke registries. The
few large series of PCA infarction described in the literature6,7
suggest that the majority of cases of PCA territory infarction are attributable
to embolic disease, while atherothrombotic lesions of the PCA are implicated in
fewer than 10% of patients.7
A number of strategies to treat
intracranial atherosclerotic stenosis are currently under evaluation,8
and this case emphasizes the need for thorough clinical and radiologic
assessment of patients with midbrain infarction. Stenosis of the PCA should be
considered as a potential aetiological factor in such patients.
References
Claude H, Loyez M.
Ramollissement du noyau rouge. Rev Neurol (Paris) 1912; 24: 49-51.
Coppola RJ, Freedman H.
Bilateral Claude syndrome: clinical and neuropathological study. Buckeye
Osteopathic Physician 1991; 60: 4-6.
Kremer C, Baumgartner RW. Aortic
Embolism in Claude’s Syndrome. Cerebrovasc Dis 2002; 13: 142-143.
Leigh RJ, Zee DS. The neurology
of eye movements, 2nd ed. Philadelphia: FA Davis, 1991.
Seo SW, Heo JH, Shin WC, Chang
DI, Kim SM, Heo K. Localization of Claude’s syndrome. Neurology 2001; 57:
2304-2307.
Steinke W, Mangold J, Schwartz
A, Hennerici M. Mechanisms of infarction in the superficial posterior
cerebral artery territory. J Neurol 1987; 21: 290-299.
Brandt T, Steinke W, Thie A,
Pessin MS, Caplan LR. Posterior Cerebral Artery Territory Infarcts: Clinical
Features, Infarct Topography, Causes and Outcome. Cerebrovasc Dis 2000; 10:
170-182.
Marks MP, Marcellus M, Norbash
AM, Steinberg GK, Tong D, Albers GW. Outcome of Angioplasty for
Atherosclerotic Intracranial Stenosis. Stroke 1999; 30: 1065-1069.