Long-term follow-up study of patients with unilateral moyamoya disease.

Although a number of cases of unilateral Moyamoya disease have been reported, the natural history of this disease remains unclear. The clinical features of 17 patients initially diagnosed with unilateral Moyamoya 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 ischemic attack was the initial episode in ten of the 12 pediatric cases, two of the five adult cases presented with intraventricular hemorrhage. Of the 12 pediatric patients six developed contralateral lesions between 4 and 34 months (mean, 20) after the diagnosis of a unilateral lesion. The remaining six pediatric patients and all adult patients did not develop lesions on the normal side. The mean age at onset for patients later developing contralateral lesions was 6.2 years. The pediatric cases remaining unilateral was 7.7 years. The normal hemisphere of three of the pediatric patients has remained unchanged on repeated follow-up angiograms for over 10 years. Young children tended to develop the vascular pathology bilaterally. However, there were some pediatric cases whose normal or atypical sides remained unchanged without development of bilateral lesions.