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[哈基姆 - 亚当斯综合征]

[Hakim-Adams syndrome].

作者信息

Shamov I A

机构信息

Dagestan State Medical Academy, Makhachkala.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2015;115(9):49-53. doi: 10.17116/jnevro20151159149-53.

Abstract

Hakim-Adams syndrome is little known, although quite common disease. The etiology is not fully clear. Pathogenetic mechanisms included the blockage of cerebrospinal fluid outflow along the upper lateral surface of the brain, development of hydrocephalus with the expansion of the brain ventricles while maintaining normotensive cerebrospinal fluid (CSF). Main manifestations are ataxia, apraxia, symptoms of dementia and urinary disturbances, up to incontinence. Diagnosis is based on clinical symptoms, exploratory lumbar and neuroimaging. Main treatment is ventriculoperitoneal (stomach) shunt placement for fluid drainage. Timely surgical intervention gives good results and improves quality of life of patients.

摘要

哈基姆-亚当斯综合征鲜为人知,尽管它是一种相当常见的疾病。其病因尚不完全清楚。发病机制包括脑脊液沿脑上外侧表面流出受阻,在脑脊液压力正常的情况下脑室扩张导致脑积水。主要表现为共济失调、失用症、痴呆症状和泌尿功能障碍,直至尿失禁。诊断基于临床症状、腰椎穿刺检查和神经影像学检查。主要治疗方法是放置脑室腹腔(胃)分流管以引流液体。及时的手术干预能取得良好效果并改善患者生活质量。

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