Hot cross bun sign (pons)
Citation, DOI, disclosures and article data
At the time the article was created Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosuresAt the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose.
View Rohit Sharma's current disclosuresThe hot cross bun sign refers to the MRI appearance of the pons when T2 hyperintensity forms a cross on axial images, representing selective degeneration of transverse pontocerebellar tracts and median pontine raphe nuclei 1.
It has been described in a variety of neurodegenerative and other conditions. Common associations include:
multiple system atrophy, cerebellar type (MSA-C) (classic sign but not pathognomonic) 1
spinocerebellar ataxia (various types) 5
Other causes which are either very rare or in which presence of the sign is rarely reported include 17:
autoimmune/paraneoplastic rhombencephalitis (e.g. anti-KLHL11, anti-Ri, anti-amphiphysin, anti-Homer-3) 7,10,14,15,16
bilateral cerebellar ischemic stroke 6
cerebral vasculitis (causing Wallerian degeneration of pontocerebellar tracts) 4
References
- 1. Prasad S & Rossi M. The Hot Cross Bun Sign: A Journey Across Etiologies. Movement Disord Clin Pract. 2022;9(8):1018-20. doi:10.1002/mdc3.13596 - Pubmed
- 2. Soares-fernandes JP, Ribeiro M, Machado A. "Hot cross bun" sign in variant Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 2009;30 (3): E37. doi:10.3174/ajnr.A1335 - Pubmed citation
- 3. Henry C, Jouan F, De Broucker T. JC virus granule cell neuronopathy: A cause of infectious cerebellar degeneration. (2015) Journal of the neurological sciences. 354 (1-2): 86-90. doi:10.1016/j.jns.2015.05.003 - Pubmed
- 4. Muqit MM, Mort D, Miskiel KA, Shakir RA. "Hot cross bun" sign in a patient with parkinsonism secondary to presumed vasculitis. (2001) Journal of neurology, neurosurgery, and psychiatry. 71 (4): 565-6. Pubmed
- 5. Y.‐C. Lee, C.‐S. Liu, H.‐M. Wu, P.‐S. Wang, M.‐H. Chang, B.‐W. Soong. The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia. (2009) European Journal of Neurology. 16 (4): 513. doi:10.1111/j.1468-1331.2008.02524.x - Pubmed
- 6. Nishida K & Futamura N. Hot Cross Bun Sign in Bilateral Middle Cerebellar Peduncle Infarction. Stroke. 2023. doi:10.1161/strokeaha.122.042453 - Pubmed
- 7. Majed M, Madhavan A, Pittock S, Dubey D. “Hot Cross Bun” Sign in a Patient With Kelch-Like Protein 11 Rhombencephalitis. Mayo Clin Proc. 2022;97(1):122-3. doi:10.1016/j.mayocp.2021.11.024 - Pubmed
- 8. Ishikawa H, Kawada N, Taniguchi A et al. Paraneoplastic Neurological Syndrome Due to Burned-Out Testicular Tumor Showing Hot Cross-Bun Sign. Acta Neurol Scand. 2015;133(5):398-402. doi:10.1111/ane.12469 - Pubmed
- 9. Ando T, Yokoi F, Riku Y et al. The Hot Cross Bun Sign in Corticobasal Degeneration. Neuropathology. 2021;41(5):376-80. doi:10.1111/neup.12745 - Pubmed
- 10. Liu Y, Huang D, Wu L. Occult Breast Cancer With Anti-Ri Antibody Positivity and Pontine Hot Cross Bun Sign. JAMA Neurol. 2023;80(2):207. doi:10.1001/jamaneurol.2022.4663 - Pubmed
- 11. Constantinides V, Paraskevas G, Kalogera S, Yapijakis C, Kapaki E. Hot Cross Bun Sign and Prominent Cerebellar Peduncle Involvement in a Patient with Oculodentodigital Dysplasia. Neurol Sci. 2020;42(1):343-5. doi:10.1007/s10072-020-04569-1 - Pubmed
- 12. Jain R, Sannegowda R, Agrawal A, Hemrajani D, Jain R, Mathur T. 'Hot Cross Bun' Sign in a Case of Cerebrotendinous Xanthomatosis: A Rare Neuroimaging Observation. Case Reports. 2013;2013(feb14 1):bcr2012006641. doi:10.1136/bcr-2012-006641 - Pubmed
- 13. Nagpal K & Agarwal P. "Hot-Cross Bun" and "Inverse Trident Sign' in Neurosarcoidosis: An Important Finding. Neurol India. 2017;65(1):175-6. doi:10.4103/0028-3886.198227 - Pubmed
- 14. Schlapakow E, Keil V, Paus M, Kornblum C, Hattingen E, Klockgether T. Multiple System Atrophy Mimicry in MRI: Watch out for Paraneoplastic Rhombencephalitis. J Clin Neurosci. 2020;76:238-40. doi:10.1016/j.jocn.2020.04.052 - Pubmed
- 15. Liu M, Ren H, Fan S et al. Neurological Autoimmunity Associated With Homer-3 Antibody. Neurol Neuroimmunol Neuroinflamm. 2021;8(6):e1077. doi:10.1212/nxi.0000000000001077 - Pubmed
- 16. Liu M, Ren H, Lin N, Tan Y, Fan S, Guan H. The “hot Cross Bun Sign” in Patients with Autoimmune Cerebellar Ataxia: A Case Report and Literature Review. Front Neurol. 2022;13:979203. doi:10.3389/fneur.2022.979203 - Pubmed
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- Multiple system atrophy cerebellar type (MSA-C)
- JC virus granule cell neuronopathy
- Multiply system atrophy cerebellar type (MSA-C)
- Multiple system atrophy (MSA-C)
- Hot cross bun sign of pons
- Multiple system atrophy (cerebellar type, MSA-C)
- Reverse hot cross bun sign
- Multiple system atrophy
- Hot cross bun sign
- JC virus granule cell neuronopathy
- Multiple system atrophy
- Olivopontocerebellar atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy
- Multiple system atrophy - cerebellar type