PDF(Pubmed)
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.
摘要：
神经退行性疾病被分类为一组具有继发于错误折叠蛋白聚集的神经元进行性丧失的疾病。这些神经退行性疾病中的一些与横桥小脑道和中桥中缝核的变性有关。这种特定的神经元变性会导致MRIT2成像上的放射学热十字头征（HCBS），并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的HCBS患病率更高，该症状也被描述为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。这里,我们介绍了一例34型脊髓小脑共济失调的病例，并提供了一个特征性的热交叉bun征，并对文献进行了简要回顾。
