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[皮质基底节变性]

[Cortico-basal degeneration].

作者信息

Morimatsu M

机构信息

Department of Neurology, Yamaguchi University School of Medicine.

出版信息

Nihon Rinsho. 1997 Jan;55(1):139-43.

PMID:9014438
Abstract

Corticobasal degeneration (CBD) is not rare disease, because in our clinic 13 patients were observed for the past 8 years, with ratio to those with Parkinson's disease being 1:18. Our clinical criteria of this disease consist of the combination of 1) limb-kinetic apraxia as cortical sign, 2) akinetic-rigid sign as extrapyramidal sign, 3) their marked asymmetry, and as additional findings, 4) the presence of grasp reflex, alien hand sign, reflex myoclonus, limb dystonia, and others, and 5) neuroimagings (MRI, SPECT) suggestive of asymmetric cortical lesions. There are reports indicating that clinical CBD was diagnosed as Pick's disease, progressive supranuclear palsy and Alzheimer's disease, pathologically. Therefore, more basic investigations, especially from molecular biology are necessary to discriminate these corticobasal complex disorders.

摘要

皮质基底节变性(CBD)并非罕见疾病,因为在我们的诊所,过去8年中观察到了13例患者,与帕金森病患者的比例为1:18。我们对该疾病的临床诊断标准包括以下几点的组合:1)作为皮质体征的肢体运动性失用症;2)作为锥体外系体征的运动不能-强直体征;3)它们的明显不对称性,以及作为附加表现的4)抓握反射、异己手征、反射性肌阵挛、肢体肌张力障碍等的存在;5)提示不对称皮质病变的神经影像学检查(MRI、SPECT)。有报告表明,临床诊断为CBD的病例在病理上曾被诊断为Pick病、进行性核上性麻痹和阿尔茨海默病。因此,需要进行更多的基础研究,尤其是来自分子生物学方面的研究,以鉴别这些皮质基底节综合征。

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