CLINICAL AND ANGIOGRAPHIC FOLLOW-UP OF CHILDHOOD-ONSET MOYAMOYA DISEASE

To clarify the differences between childhood-onset moyamoya disease an d that with onset in adulthood, we studied the clinical course and ang iographic findings of adult patients (over 20 years of age) with moyam oya disease of childhood onset (up to 15 years of age). The clinical c ourse in 25 patients could be assessed. The follow-up period was 5-27 years. Neurological deficits were noted in 11/23 and mental disorders in 9/21. In all except one, the illness had started before the age of 7 years. Neither neurological nor mental condition changed during or a fter adolescence (15-20 years of age). Two patients died of intracrani al hemorrhage. The disease progressed in angiographic stage until adol escence, but had stabilized or almost stabilized by the age of 20 year s. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiogra phic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.