[Moyamoya disease: Diagnosis, clinical features, evolution and treatment in 10 patients]

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.

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.

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.

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.