关键词: hot cross bun sign multiple system atrophy (MSA) spinocerebellar ataxia type 2 and 7

来  源:   DOI:10.1002/mdc3.13550   PDF(Pubmed)

Abstract:
UNASSIGNED: The \"hot cross bun\" sign is a cruciform hyperintensity is seen on T2 weighted imaging within the pons. The sign is considered to be pathognomic for Multiple system atrophy type C. The clinical and radiological features of Multiple system atrophy type C overlap with the autosomal dominant inherited ataxias. We present a case series of 3 African patients with genetically proven Spinocerebellar Ataxia presenting with the Hot cross bun sign and a scoping review of similar studies.
UNASSIGNED: We described the phenotypic and radiological presentation of genetically confirmed SCA-2 in two, and SCA-7 in one patient, with the \"hot cross bun\" sign.
UNASSIGNED: We performed a scoping review on the Hot Cross Bun Sign.A total of 66 articles were retrieved. We describe the diverse aetiologies of the sign and associated phenotypic and radiological features. We review the Spinocerebellar Ataxias described with a Hot cross bun sign and make comparisons to Multiple System Atrophy Type C [Ref. 1,2].
UNASSIGNED: To our knowledge this is the first description of an African cohort presenting with the Hot Cross Bun Sign. We expand the differential diagnosis of the Hot Cross Bun Sign.
摘要:
“热十字bun”标志是在脑桥内的T2加权成像上看到的十字形高强度。该体征被认为是多系统萎缩C型的病因学特征。多系统萎缩C型的临床和放射学特征与常染色体显性遗传性共济失调重叠。我们介绍了3例经过遗传证实的脊髓小脑共济失调的非洲患者的病例系列,这些患者表现为Hotcrossbun标志,并对类似研究进行了范围审查。
我们描述了两种经遗传证实的SCA-2的表型和放射学表现,和一名患者的SCA-7,带有“热十字面包”标志。
我们对HotCrossBun标志进行了范围审查。共检索到66篇文献。我们描述了该标志的多种病因以及相关的表型和放射学特征。我们回顾了用HotCross标志描述的脊髓小脑共济失调,并与C型多系统萎缩进行了比较[Ref.1,2].
据我们所知,这是对带有HotCrossBun标志的非洲队列的首次描述。我们扩展了热十字面包征的鉴别诊断。
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