CLINICAL AND ANGIOGRAPHIC FOLLOW-UP OF CHILDHOOD-ONSET MOYAMOYA DISEASE
CLINICAL AND ANGIOGRAPHIC FOLLOW-UP OF CHILDHOOD-ONSET MOYAMOYA DISEASE
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DOI：
10.1007/bf00300998
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发表时间：
1995-10-01
影响因子：
1.4
通讯作者：
MIZOI, K
中科院分区：
文献类型：
--
作者：
EZURA, M;YOSHIMOTO, T;MIZOI, K
为了明确儿童期发病的烟雾病与成年期发病的烟雾病的区别，我们研究了儿童期发病的烟雾病（15岁以下）成人患者（20岁以上）的临床病程和血管造影结果。对25例患者的临床病程进行评估。随访5 ~ 27年。11/23出现神经功能障碍，9/21出现精神障碍。除一人外，其他病例都是在7岁之前发病的。在青春期（15-20岁）期间或之后，神经和精神状况都没有改变。2例患者死于颅内出血。该疾病在血管造影阶段进展到青春期，但在20岁时稳定或几乎稳定。本研究提示儿童期发病的烟雾病具有较高的发病率和死亡率。该疾病在儿童和青少年之间的血管造影阶段进展，但在青少年和成年之间稳定或几乎稳定。
To clarify the differences between childhood-onset moyamoya disease and that with onset in adulthood, we studied the clinical course and angiographic findings of adult patients (over 20 years of age) with moyamoya disease of childhood onset (up to 15 years of age). The clinical course 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 after adolescence (15-20 years of age). Two patients died of intracranial hemorrhage. The disease progressed in angiographic stage until adolescence, but had stabilized or almost stabilized by the age of 20 years. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiographic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.