LONG-TERM FOLLOW-UP-STUDY OF PATIENTS WITH UNILATERAL MOYAMOYA-DISEASE

Although a number of cases of unilateral Moyamoya disease have been re ported, the natural history of this disease remains unclear. The clini cal features of 17 patients initially diagnosed with unilateral Moyamo ya disease at our hospital are reported. Age at onset was 3-45 years ( mean, 13.5). Of these 12 cases had onset of symptoms in childhood and five had onset in adulthood. Seven were male and 10 were female. An is chemic attack was the initial episode in ten of the 12 pediatric cases , two of the five adult cases presented with intraventricular hemorrha ge. Of the 12 pediatric patients six developed contralateral lesions b etween 4 and 34 months (mean, 20) after the diagnosis of a unilateral lesion. The remaining six pediatric patients and all adult patients di d not develop lesions on the normal side. The mean age at onset for pa tients later developing contralateral lesions was 6.2 years. The pedia tric cases remaining unilataral was 7.7 years. The normal hemisphere o f three of the pediatric patients has remained unchanged on repeated f ollow-up angiograms for over 10 years. Young children tended to develo p the vascular pathology bilaterally. However, there were some pediatr ic cases whose normal or atypical sides remained unchanged without dev elopment of bilateral lesions. (C) 1997 Elsevier Science B.V.