Moyamoya disease: advantage of early diagnosis and surgical treatment

A diagnosis of moyamoya disease was made in three children aged five, eight and 13 years (including two Turkish sisters). Clinical presentation was re current episodes of cerebral ischemia and stroke. CT scans and MRI showed i nfarcts in various distributions. Angiograpy revealed anterior bilateral st enosis of the circle of Willis and development of Moyamoya collateral pathw ays. In one case there was coagulopathy with protein C deficiency. To incre ase transdural collateral flow, revascularisation with encephalo-duro-arter io-synangiosis was attempted in all three children. Outcome was clinically and angiographically satisfactory and none of the children developed furthe r neurological complications. The current state of study on Moyamoya diseas e is also presented. (C) 1999 Editions scientifiques et medicales Elsevier SAS.