Moyamoya disease: a report of four cases of Thai children.

ABSTRACT

Moyamoya disease is a cerebrovascular disorder characterized by occlusion of the internal carotid arteries with cerebral angiographic finding of smoke-like collateral vessels. Typical moyamoya blush can also be seen in immediate enhanced CT scan of the brain. The disease is rare in non Japanese. The present communication reports four Thai children aged 3-12 years, presented at the Department of Pediatrics, Ramathibodi hospital with recurrent hemiparesis. In one patient, brain biopsy was almost carried out. The disease should be suspected in every child with recurrent cerebral ischemic episodes. Cerebral angiogram and immediate enhanced CT scan are the two most useful investigation procedures. Either of them should be selected first for diagnosis before other unnecessary invasive investigations are done.