[Moyamoya disease. A cause of vascular occlusion in childhood]

The objective of this study was to review the cases of Moya-Moya diagnosed in our center.

We reviewed the Moya-Moya cases diagnosed from 1979 to 1997. We evaluated the following elements: age of clinical onset, sex, clinical features, complementary examinations, neuroimage, treatment and follow-up.

Six patients were diagnosed. The first case appeared in 1979 and the last in 1997. These included four boys and 2 girls, with ages between 5 months and 14 years. The initial clinical feature in all six cases was acute hemiparesis, noting that in one case this was preceded by homolateral seizures. Neuroimaging revealed ischaemic infarction areas in brain CT or MNR. The diagnosis was based on angiography, where in four cases there appeared bilateral occlusion of the internal carotid, or of the anterior or middle cerebral arteries and in the other two there was a unilateral occlusion of the interior carotid and middle cerebral arteries. Regarding etiology, in four patients the dysfunction was due to either fibromuscular dysplasia of the carotid, neurofibromatosis, cranial trauma or to Down's syndrome. In the other two cases no other primary cause was found.

Acute stroke is an infrequent disease of pediatric age patients, however it is necessary to do a thorough angiography study to rule out the Moya-Moya like vascular anomalies.