Department of Neurology, University Hospital of Bonn, Sigmund-Freud-Strasse 25, 53127 Bonn, Germany.
Department of Neuroradiology, University Hospital of Bonn, Sigmund-Freud-Strasse 25, 53127 Bonn, Germany.
J Clin Neurosci. 2020 Jun;76:238-240. doi: 10.1016/j.jocn.2020.04.052. Epub 2020 Apr 16.
The hot cross bun (HCB) sign describes cruciform-shaped T2-weighted hyperintensities of pontocerebellar fibers within the pons and is a typical, but not specific imaging hallmark of the cerebellar variant of multiple system atrophy (MSA-C). We report a case of a 51-year-old woman who was first diagnosed with MSA-C based on progressive cerebellar ataxia, the HCB sign and T2-weighted hyperintensities in middle cerebellar peduncles on MRI. However, further diagnostic work-up revealed positive anti-amphiphysin antibodies in blood and cerebrospinal fluid and subsequently breast cancer. This report of a paraneoplastic rhombencephalitis which initially mimicked MSA-C imaging features stresses the importance of considering immune-mediated rhombencephalitis as differential diagnosis in cases of progressive cerebellar ataxia and the HCB sign on T2-weighted MRI, especially in the absence of pontocerebellar atrophy.
热十字面包征（HCB 征）描述桥脑小脑纤维内十字形 T2 加权高信号，是多系统萎缩（MSA-C）小脑变异型的典型但非特异性影像学标志。我们报告了一例 51 岁女性，最初根据进行性小脑共济失调、HCB 征和 MRI 中中脑小脑脚 T2 加权高信号诊断为 MSA-C。然而，进一步的诊断检查显示血液和脑脊液中存在抗 Amphiphysin 抗体，随后发现乳腺癌。本例副肿瘤性脑桥脑炎最初类似于 MSA-C 的影像学特征，强调了在进行性小脑共济失调和 T2 加权 MRI 上出现 HCB 征的情况下，尤其是在没有桥脑小脑萎缩的情况下，将免疫介导性脑桥脑炎作为鉴别诊断的重要性。
