Naidoo Ansuya Kasavelu, Wells Cait-Lynn Deanne, Rugbeer Yashvir, Naidoo Neil
Greys Academic Hospital Pietermaritzburg South Africa.
School of Clinical Medicine, Division Neurology University of KwaZulu Natal Durban South Africa.
Mov Disord Clin Pract. 2022 Oct 13;9(8):1105-1113. doi: 10.1002/mdc3.13550. eCollection 2022 Nov.
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.
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.
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].
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.
“热十字面包”征是指在脑桥的T2加权成像上出现的十字形高信号。该征象被认为是C型多系统萎缩的特征性表现。C型多系统萎缩的临床和影像学特征与常染色体显性遗传性共济失调重叠。我们报告了3例具有基因证实的脊髓小脑共济失调的非洲患者的病例系列，这些患者均表现出“热十字面包”征，并对类似研究进行了综述。
我们描述了2例基因确诊的SCA - 2患者和1例SCA - 7患者的表型和影像学表现，他们均有“热十字面包”征。
我们对“热十字面包”征进行了综述。共检索到66篇文章。我们描述了该征象的多种病因以及相关的表型和影像学特征。我们回顾了描述有“热十字面包”征的脊髓小脑共济失调，并与C型多系统萎缩进行比较[参考文献1,2]。
据我们所知，这是首次对出现“热十字面包”征的非洲队列进行描述。我们扩展了“热十字面包”征的鉴别诊断。
