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一篇关于橄榄体脑桥小脑萎缩的致歉与介绍。

An apology and an introduction to the olivopontocerebellar atrophies.

作者信息

Duvoisin R C

出版信息

Adv Neurol. 1984;41:5-12.

PMID:6496230
Abstract

The OPCAs are not, as Dejerine and Thomas (6) and subsequent writers have suggested, a unitary morbid entity. They comprise a heterogeneous group of disorders sharing certain major clinical and morphologic features with the hereditary and acquired ataxias and with the extrapyramidal system disorders, such as Parkinson's disease and the hereditary and acquired choreas, yet distinct from both these general groups of neurodegenerative diseases. Some of the OPCAs are sharply defined morbid entities of known etiology identified on combined genetic and clinical grounds or by a unique biochemical defect; most are of uncertain provenance and may themselves represent a collection of multiple, as yet undetermined entities. The nosologic concept of OPCA survives today in part because of its (Formula: see text) clinical usefulness in categorizing patients with mixed cerebellar and extrapyramidal syndromes and its importance in the differential diagnosis of both extrapyramidal and cerebellar disorders.

摘要

橄榄体脑桥小脑萎缩(OPCAs)并不像德热里纳(Dejerine)和托马斯(Thomas)(6)以及后来的作者所认为的那样,是一种单一的病态实体。它们是一组异质性疾病,与遗传性和获得性共济失调以及锥体外系疾病(如帕金森病、遗传性和获得性舞蹈病)具有某些共同的主要临床和形态学特征,但又不同于这两组神经退行性疾病。一些OPCAs是基于遗传学和临床综合依据或独特的生化缺陷而明确界定的已知病因的病态实体;大多数OPCAs的病因不明,可能本身就代表了多种尚未确定的实体的集合。OPCAs的疾病分类概念如今仍然存在,部分原因在于它在对伴有小脑和锥体外系混合综合征的患者进行分类方面具有临床实用性,以及在锥体外系和小脑疾病的鉴别诊断中具有重要意义。

相似文献

1
An apology and an introduction to the olivopontocerebellar atrophies.一篇关于橄榄体脑桥小脑萎缩的致歉与介绍。
Adv Neurol. 1984;41:5-12.
2
Clinical features of sporadic (Dejerine-Thomas) olivopontocerebellar atrophy.散发性(德热里纳 - 托马斯型)橄榄脑桥小脑萎缩的临床特征。
Adv Neurol. 1984;41:217-24.
3
[Clinical features of "multiple system atrophies"--olivo-ponto-cerebellar atrophy, striato-nigral degeneration and Shy-Drager syndrome].
Rinsho Shinkeigaku. 1985 Oct;25(10):1196-203.
4
[Cerebellar atrophies].[小脑萎缩]
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Dominant olivopontocerebellar atrophy with dementia and extrapyramidal signs: report of a family through three generations.伴有痴呆和锥体外系症状的显性橄榄体脑桥小脑萎缩:一个三代家系报告
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[Striatonigral degeneration and sporadic olivopontocerebellar atrophy: a consideration of the clinical entity of multiple system atrophy].[纹状体黑质变性与散发性橄榄脑桥小脑萎缩:对多系统萎缩临床实体的思考]
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Olivopontocerebellar atrophy: toward a better nosological definition.橄榄体脑桥小脑萎缩:迈向更完善的疾病分类学定义
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[Neuroradiologic and neurophysiologic studies in patients with olivo-ponto-cerebellar atrophy].橄榄体脑桥小脑萎缩患者的神经放射学和神经生理学研究
Riv Neurobiol. 1984 Apr-Sep;30(2-3):575-89.

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Increased intrinsic membrane excitability is associated with hypertrophic olivary degeneration in spinocerebellar ataxia type 1.内在膜兴奋性增加与1型脊髓小脑共济失调中的肥大性橄榄核变性相关。
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Multiple system atrophy--the nature of the beast.
多系统萎缩——疾病的本质
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Cytoplasmic argyrophilic inclusions in neurons of pontine nuclei in patients with olivopontocerebellar atrophy: immunohistochemical and ultrastructural studies.橄榄体脑桥小脑萎缩患者脑桥核神经元中的胞质嗜银包涵体:免疫组织化学和超微结构研究
Acta Neuropathol. 1990;79(6):584-94. doi: 10.1007/BF00294235.