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Lumbar Puncture Alleviates Chorea in a Patient with Huntington’s Disease and Normal Pressure Hydrocephalus
Behavioural Neurology ( IF 2.8 ) Pub Date : 2009 , DOI: 10.3233/ben-2009-0239
Peyman Shirani, Alicia R. Salamone, Elham Lahijani, Michele K. York, Paul E. Schulz

A 44-year-old African-American male was admitted to our hospital after a suicide attempt. He had depression, poor cognitive function, choreiform movements, difficulty pronouncing words, and difficulty walking. His symptoms had worsened markedly over several months. Chorea lead to genetic testing that confirmed a diagnosis of Huntington Disease (HD). A CT scan of the head showed wider ventricles than is typical of HD. The head CT and gait change suggested normal pressure hydrocephalus (NPH). Lumbar puncture (LP) led to improved neuropsychologic test scores and walking thereby supporting the diagnosis of NPH. Surprisingly, the LP also led to an 80% improvement of chorea. There are two other reports of an association between HD and NPH. NPH should be considered in HD patients with atypical symptoms, such as the inability to walk or rapid progression, as its treatment may lead to improved cognition, gait, and chorea.

中文翻译:

腰椎穿刺缓解亨廷顿舞蹈病和常压性脑积水患者的舞蹈病

自杀未遂,一名44岁的非洲裔美国男性被送进我们医院。他患有抑郁症,认知功能差,舞蹈形式的动作,发音困难和行走困难。在过去的几个月中,他的症状明显恶化。舞蹈病导致的基因检测证实了亨廷顿舞蹈病(HD)的诊断。头部的CT扫描显示脑室比HD的宽。头部CT和步态改变提示正常压力脑积水(NPH)。腰椎穿刺(LP)可以改善神经心理学测验分数和步行能力,从而支持NPH的诊断。令人惊讶的是,LP也使舞蹈症的病情改善了80%。HD和NPH之间存在关联的其他两个报告。在非典型症状的HD患者中应考虑NPH,例如无法行走或快速发展,
更新日期:2020-09-25
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