Article

Moyamoya disease is a rare chronic cerebrovascular disease. The
objective of this article is to report the different clinical and radiological
presentations and describe treatments and clinical course of this disease.
METHODS: We searched for patients with Moyamoya disease diagnosed at a French
university hospital. The diagnosis was based on arteriographic records showing
uni- or bilateral stenosis of distal intracranial internal carotid arteries or
middle cerebral arteries associated with a classic collateral network imparting a
puff of smoke aspect. Data about clinical and radiological symptoms were analyzed
for all identified patients. RESULTS: Ten patients were recorded between 2009 and
2014 including one child and nine adults. The initial presentation was
intracerebral hemorrhage in two patients, ischemic stroke in six, and either
exercice-related transient ischemic attacks or syncope in two. Recurrent events
were noted in four patients. Four patients had one or several recurrent vascular
events. Eight patients were given medical treatment and none underwent surgery.
Secondary Moyamoya syndrome was suspected in two patients, all the others one
were considered idiopathic. CONCLUSION: Moyamoya disease is a rare but
potentially severe illness. The initial presentation is more frequently an
ischemic stroke; recurrences are frequent. The diagnosis is based on
arteriography, which is also recommended to search for a cause.
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Langue : FRANCAIS