Spinocerebellar ataxia-type 34: A case report and brief review of the literature

Document Type

Journal Article

Publication Date

11-1-2023

Journal

Radiology case reports

Volume

18

Issue

11

DOI

10.1016/j.radcr.2023.08.055

Keywords

Hot cross bun sign; Multiple system atrophy; Neurodegenerative disorders; SCA-34; Spinocerebellar ataxia

Abstract

Neurodegenerative disorders are classified as a group of diseases with progressive loss of neurons secondary to aggregation of misfolded proteins. A few of these neurodegenerative diseases have been associated with degeneration of the transverse pontocerebellar tracts and median pontine raphe nuclei. This specific neuron degeneration results in the radiologic hot cross bun sign (HCBS) on MRI T2 imaging and helps narrow down the differential diagnosis. While multiple system atrophy has a higher prevalence of the HCBS than other neurodegenerative diseases, the sign has also been described with other neurodegenerative disorders such as spinocerebellar ataxia (SCA), and variant Creutzfeldt-Jakob disease. Here, we present a case of spinocerebellar ataxia type 34 with a characteristic hot-cross bun sign and provide a brief review of the literature.

Department

School of Medicine and Health Sciences Student Works

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