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“Hot Cross Bun” Sign in a Patient with Glutamic Acid Decarboxylase 65-KDa Isoform Associated Cerebellar Ataxia: Case Report and Review of the Literature

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Abstract

The “hot cross bun” sign (HCBs) is a cruciform hyperintensity on T2-weighted imaging within the pons initially found in patients diagnosed as multiple system atrophy. However, recent findings have broadened the disease spectrum presented with HCBs. Here is a case report at an academic medical center. Cerebral magnetic resonance imaging (MRI), electroneuromyography, serum, and CSF analysis were performed. Literature is comprehensively reviewed. We investigated a woman presented with blurred speech and cerebellar ataxia. Her MRI showed the vertical line of HCBs 2 weeks after disease onset and gradually enhanced, presenting as an intact HCBs in a year. Glutamic acid decarboxylase 65-kDa isoform (GAD65) antibody IgG was detected in serum and CSF. The patient was diagnosed as GAD65 associated cerebellar ataxia and treated with corticosteroid and rituximab. We found 6 previously reported autoimmune cerebellar ataxia patients with HCBs. Anti-KLHL-11, anti-Homer-3, anti-Ri, and anti-Amphiphysin were associated. All patients had cerebellar ataxia with other neurological symptoms. Five patients were diagnosed with tumor. First-line immunotherapy including corticosteroid, intravenous immunoglobulin, and plasma exchange for most patients was unsatisfied. This case highlights the importance of considering GAD65 IgG evaluation in patients with progressive cerebellar syndrome and HCBs. Early diagnosis and therapy are challenging but imperative. Further studies are required in regard to therapeutic management.

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Data will be shared by the corresponding author upon reasonable request.

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Acknowledgements

We thank the patient for granting permission to publish this information.

Funding

This work is supported by National Natural Science Foundation of China (No. 82101487), Natural Science Foundation of Shandong Province, China (ZR2021QH161), and Taishan Scholar Program of Shandong Province (tsqn202211318).

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Authors and Affiliations

  1. Department of Neurology, Cheeloo College of Medicine, Qilu Hospital of Shandong Univeristy, Jinan, 250012, Shandong, China

    Ruo-Nan Duan & Li-Li Cao

  2. Department of Radiology, Qilu Hospital of Shandong Univeristy, Jinan, Shandong, China

    Jing-Zhen He

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  1. Ruo-Nan Duan
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Contributions

Ruo-Nan Duan: Conceptualization, Data Curation, Formal Analysis, Funding Acquisition, Writing – Original Draft Preparation

Jing-Zhen He: Formal Analysis

Li-Li Cao: Conceptualization, Writing – Review & Editing

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Correspondence to Li-Li Cao.

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Duan, RN., He, JZ. & Cao, LL. “Hot Cross Bun” Sign in a Patient with Glutamic Acid Decarboxylase 65-KDa Isoform Associated Cerebellar Ataxia: Case Report and Review of the Literature. Cerebellum (2023). https://doi.org/10.1007/s12311-023-01631-4

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