FAMILIAL OCCURRENCE OF MOYAMOYA-DISEASE

There is extensive evidence that Moyamoya disease has a tendency for m ultifactorial inheritance, although the pathogenesis of Moyamoya disea se is not clear. The authors report five cases showing familial occurr ence of Moyamoya disease and analyse its clinical characteristics. In the past 15 years, we have encountered 68 cases of Moyamoya disease. A mong these, 14 cases (10 females and four males, five family pedigrees , asymptomatic 1 case) of familial occurrence were observed. In this s eries, mother-to-child inheritance was observed in five cases, althoug h there were no cases showing father-to-child inheritance. Ten patient s were children with an initial onset of cerebral ischemia, at a mean age of 9.7 years. One mother was asymptomatic and two mothers had a pa st history of cerebral ischemia. Only one patient was a 37-year-old wo man with clinical onset of intracerebral hemorrhage. There were no spe cific clinical characteristics in familial Moyamoya disease compared w ith those in sporadic Moyamoya disease. (C) 1997 Elsevier Science B.V.