Batheja Vivek, Fish Morgan, Balar Aneri B, Hedge Siddhi, Hogg Jeffery P, Lakhani Dhairya A, Khan Musharaf
Department of Medicine, George Washington University Hospital, Washington, DC, USA.
Department of Radiology, West Virginia University, Morgantown, WV, USA.
Radiol Case Rep. 2023 Aug 30;18(11):3954-3958. doi: 10.1016/j.radcr.2023.08.055. eCollection 2023 Nov.
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.
神经退行性疾病被归类为一组由于错误折叠蛋白聚集导致神经元进行性丧失的疾病。其中一些神经退行性疾病与桥脑小脑横束和脑桥中缝核的变性有关。这种特定的神经元变性在MRI T2成像上表现为放射学上的“热交叉面包征”（HCBS），有助于缩小鉴别诊断范围。虽然多系统萎缩比其他神经退行性疾病更易出现HCBS，但该征象也见于其他神经退行性疾病，如脊髓小脑共济失调（SCA）和变异型克雅氏病。在此，我们报告一例具有特征性热交叉面包征的34型脊髓小脑共济失调病例，并对相关文献进行简要综述。
