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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.

Titel:	"Hot Cross Bun" Sign in a Patient with Glutamic Acid Decarboxylase 65-KDa Isoform Associated Cerebellar Ataxia: Case Report and Review of the Literature.
Autor/in / Beteiligte Person:	Duan, RN ; He, JZ ; Cao, LL
Link:	View record at Springer (Volltext)
Zeitschrift:	Cerebellum (London, England), Jg. 23 (2024-06-01), Heft 3, S. 1204-1210
Veröffentlichung:	<2006->: New York : Springer ; <i>Original Publication</i>: London : Martin Dunitz, c2002-, 2024
Medientyp:	academicJournal
ISSN:	1473-4230 (electronic)
DOI:	10.1007/s12311-023-01631-4
Schlagwort:	Humans Female Magnetic Resonance Imaging Middle Aged Autoantibodies blood Autoantibodies immunology Glutamate Decarboxylase immunology Cerebellar Ataxia diagnostic imaging
Sonstiges:	Nachgewiesen in: MEDLINE Sprachen: English Publication Type: Journal Article; Case Reports; Review Language: English [Cerebellum] 2024 Jun; Vol. 23 (3), pp. 1204-1210. <i>Date of Electronic Publication: </i>2023 Nov 10. MeSH Terms: Glutamate Decarboxylase* / immunology ; Cerebellar Ataxia* / diagnostic imaging ; Humans ; Female ; Magnetic Resonance Imaging ; Middle Aged ; Autoantibodies / blood ; Autoantibodies / immunology References: Shrivastava A. The hot cross bun sign. Radiology. 2007;245:606–7. https://doi.org/10.1148/radiol.2452041856 . (PMID: 10.1148/radiol.245204185617940314) ; Lee YC, Liu CS, Wu HM, Wang PS, Chang MH, Soong BW. The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia. Eur J Neurol. 2009;16:513–6. https://doi.org/10.1111/j.1468-1331.2008.02524.x . (PMID: 10.1111/j.1468-1331.2008.02524.x19187260) ; Horimoto Y, Aiba I, Yasuda T, Ohkawa Y, Katayama T, Yokokawa Y, Goto A, Ito Y. 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(PMID: 10.1186/s12883-020-01738-9323406087184719) ; Zhu S, Deng B, Huang Z, Chang Z, Li H, Liu H, Huang Y, Pan Y, Wang Y, Chao YX, Chan LL, Wu YR, Tan EK, Wang Q. “Hot cross bun” is a potential imaging marker for the severity of cerebellar ataxia in MSA-C. NPJ Parkinsons Dis. 2021;7:15. https://doi.org/10.1038/s41531-021-00159-w . (PMID: 10.1038/s41531-021-00159-w335896307884406) Contributed Indexing: Keywords: Autoimmune cerebellar ataxia; GAD65; HCBs; Paraneoplastic syndromes Substance Nomenclature: 0 (glutamate decarboxylase 2) Entry Date(s): Date Created: 20231110 Date Completed: 20240518 Latest Revision: 20240518 Update Code: 20240518

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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.