Familial occurrence of moyamoya disease. Magnetic resonance angiography as a screening test for high-risk subjects

Abstract

The authors report four cases of familial occurrence of moyamoya disease. Although the pathogenesis of moyamoya disease is not clear, there is extensive evidence that this disease has a tendency to show multifactorial inheritance. Therefore, a screening test for those at high risk, i.e., who have a moyamoya patient among their blood relatives, is clinically important. Magnetic resonance angiography (MRA) successfully revealed abnormal findings specific to moyamoya disease in members of the four probands families. MRA is a powerful and noninvasive way of detecting individuals at high risk of developing moyamoya disease.