Pontine hot cross bun sign in spinocerebellar ataxia type 2

Soto, María Carolina Sepúlveda; Munhoz, Renato P.

doi:10.1590/0004-282x20200066

Cited by 1 publication

(1 citation statement)

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“…Laboratory testing for infective, autoimmune and metabolic disorders and CSF examination including Varicella Zoster Virus, Herpes Simplex Virus, Cytomegalovirus and John Cunningham Virus (JCV) PCR were normal. Her CD4 count was [6][7][8]18,44,52,[84][85][86][87] Probable Dementia with Lewy Body 1 15 SCA 3 9 6,12,44,3,5,84,86,88,89 Corticobasal Degeneration (olivopontocerebellar type) 1 14 SCA 6, 7, 8 17 4,6,44,47,84,86,90,91 Parkinsonism Related Vasculitis 1 16 SCA 10 1 84 Infection SCA 17 1 10 Rabies Encepaltiis 1 2 SCA 23 1 92 HIV related Progressive Multifocal Leukoencephalopathy 4 22,23,25,58 SCA 31 1 47 Natalizumab associated Progressive Multifocal Leukoencephalopathy 1 29 SCA 34 4 13,86,93,94 HIV related JCV granule cell neuronopathy 1 60 SCA 42 1 95 HIV 2 27,…”

Section: Casementioning

confidence: 99%

The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature

Naidoo

Wells

Rugbeer

et al. 2022

Movement Disord Clin Pract

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Background 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. Cases 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. Literature Review 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]. Conclusions 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.

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