Background
The clinical course of Moyamoya disease (MMD) is different in pediatric and adult patients. MMD seemed to progress with aging in childhood and then stabilized in adulthood. However, a few reports have indicated the dynamic angiographic change even in adult-onset MMD. Thus, in this study, we reviewed adult cases with progress in angiographic stage and report our case of adult-onset MMD with progression confirmed by cerebral angiography.
Methods
We reviewed the clinical and radiographic records obtained in previous published reports of adult-onset (over 20 years) MMD with progression. This study encompassed only 19 cases; our case and 18 which were previously reported in the literature. We could divide these cases to two groups by angiographic appearance (unilateral or bilateral lesion at diagnosis). In these two groups, there was subgroup in which progression was found after surgical procedure. Angiographic stage was graded using Suzuki's classification.
Results
The age at onset was 21–68 years (mean, 39. 8 ± 13.3) with age peaks in third decade. Females were more commonly involved (78.6%) than males. Ischemic stroke was the most common initial presentation, and there were only two cases with hemorrhagic stroke. The mean period until follow-up angiography was 35.3 months, and almost cases (84.2%) have advanced stage within 5 years. Moreover, about half (42.9%) of patients were asymptomatic, when angiographic progression was confirmed. Our case was a symptomatic 43-year-old Japanese woman with right hemispheric TIA. There was no familial and medical history. Cerebral angiography revealed MMD (stage III bilaterally according to Suzuki). Antiplatelet medication with aspirin (100 mg/day) was begun. When she was 45 years old, she was readmitted to our hospital for surgical treatment because of repeated ischemic episodes. Cerebral angiography demonstrated the progressive appearance (stage V bilaterally).
Conclusions
To our knowledge, our study is the first review for progressive adult-onset moyamoya disease in the English-language literature. There seem to be more cases with adult-onset MMD with progression than previously report. Our review indicates that ‘adult-onset MMD with progression’ includes the several different pathological patterns. We suggested that careful observation for at least 5 years is required even if in adulthood.