Clinical features of familial moyamoya disease in China / 国际脑血管病杂志

ABSTRACT

Objective To investigate the clinical features of familial moyamoya disease in China. Methods The patients w ith familial moyamoya disease admitted to the department of Neurosurgery, the 307th Hospital of PLA from March 2009 to June 2012 w ere analyzed retrospectively. Results Of 1 108 patients w ith moyamoya disease admitted to the department of Neurosurgery, the 307th Hospital of PLA, 87 patients (7.8%) w ith familial moyamoya disease w ere identified. Familial moyamoya disease w as observed both in the Han nationality and the minority nationalities. The male to female ratio w as 1∶1.02. The age at first onset ranged from 8 months to 59 years. There w ere tw o peak ages -of-onset, 5-9 years and 30-34 years, respectively). The most common initial symptom w as cerebral ischemia (74 .7%). The first degree relatives w ere the most affected in patients w ith familial moyamoya disease (78/87, 89 .66%), in w hich the siblings accounted for most of the disease ( 38/78, 48.72%), and the proportions of mother-to-child inheritance (21/78, 26.92%) and father-to-child inheritance ( 19/78, 24.36%) w ere similar. Conclusions There are tw o peak ages-of-onset, cerebral ischemia is the most common initial symptom, the first degree relatives are the most affected in patients w ith familial moyamoya disease in China.