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“热十字面包”征并非总是多系统萎缩:11例病因分析

The 'Hot Cross Bun' Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases.

作者信息

Way Christopher, Pettersson David, Hiller Amie

机构信息

Parkinson's Institute and Clinical Center, Sunnyvale, CA, USA.

Department of Diagnostic Radiology, Neuroradiology, Oregon Health & Sciences University, Portland, OR, USA

出版信息

J Mov Disord. 2019 Jan;12(1):27-30. doi: 10.14802/jmd.18031. Epub 2018 Dec 19.

Abstract

OBJECTIVE

To clarify the specificity of the 'hot cross bun' sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.

METHODS

The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords 'hot cross bun,' 'pontocerebellar,' 'cruciate,' 'cruciform,' 'MSA,' 'multiple system atrophy,' and 'multisystem atrophy.' Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient's neurologic symptoms.

RESULTS

Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11).

CONCLUSION

MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

摘要

目的

阐明“热十字面包”征(HCBS)在成人小脑性共济失调或帕金森综合征中对多系统萎缩(MSA)的特异性。

方法

利用关键词“热十字面包”“脑桥小脑”“十字形”“十字状”“MSA”“多系统萎缩”和“多系统萎缩”查询学术中心及附属退伍军人医院的放射信息系统。由一名神经科医生和一名神经放射科医生对扫描结果进行审查以识别HCBS。对有HCBS的受试者由两名神经科医生进行审查以确定患者神经症状最可能的病因。

结果

共识别出11例。病因包括MSA(4例可能,2例可能)、遗传性小脑性共济失调(3/11)、可能的路易体痴呆(1/11)以及尸检后仍不明确(1/11)。

结论

MSA是最常见的病因。然而,11例患者中有5例并非MSA。最常见的其他病因是未明确的遗传性小脑性共济失调(3/11)。

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