Seong-Beom Koh; Kun-Woo Park; Dae-Hie Lee

doi:10.14802/jmd.08022

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Neuroimages Cruciform Pontine MRI Hyperintensities (“Hot Cross Bun” Sign) in Non-Multiple System Atrophy Patients: Seong-Beom Koh, MD, Kun-Woo Park, MD, Dae-Hie Lee, MD; Journal of Movement Disorders 2008;1(2):107-108.
DOI: https://doi.org/10.14802/jmd.08022
Published online: October 30, 2008

Department of Neurology, Korea University College of Medicine, Seoul, Korea

Address for correspondence: Seong-Beom Koh, MD, PhD, Department of Neurology, Korea University College of Medicine at Guro Hospital, 80 Guro 2-dong, Guro-gu, Seoul, 152-703, Korea, Tel: +82-2-2626-1250, Fax: +82-2-2626-1255, E-mail: parkinson@korea.ac.kr

• Received: October 24, 2007 • Accepted: May 19, 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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A 20-year-old woman presented with a 5-year history of gait disturbance and tremor. Her mother showed the same clinical features and expired due to aspiration pneumonia at the age of 28-year-old. She was diagnosed as a spinocerebellar ataxia type 2 with molecular genetic PCR analysis. Brain T2-weighted MR image showed cruciform signal hyperintensity in pons (Figure A). A 61-year-old man presented with a 7-year history of gait disturbance after cerebellar hemorrhage. He did not showed autonomic dysfunction. Brain T2-weighted MR image showed cruciform signal hyperintensity in pons (Figure B). Cruciform pontine MRI hyperintensities (“hot cross bun” sign) is a radiologic sign which has been said to specific for multiple system atrophy.[^1,²] But our patients were diagnosed as spinocerebellar ataxia type 2 and old cerebellar hemorrhage. Therefore we suggest that “hot cross bun” sign reflects degeneration of transverse pontocerebellar fibers and is not a pathognomic sign of multiple system atrophy.

Figure.

(A) Cruciform signal hyperintensities within the pons and atrophy of pons are demonstrated on the axial T2-weighted MRI of 20-year-old spinocerebellar atrophy type 2 patient with a 5-year history of ataxia. (B) Cruciform signal hyperintensities within the pons and atrophy of pons are demonstrated on the axial T2-weighted MRI of 61-year-old patient with a 7-year history of cerebellar hemorrhage.

REFERENCES

1. Schrag A, Good CD, Miszkiel K, Morris HR, Mathias CJ, Lees AJ, et al. Differentiation of atypical parkinsonian syndromes with routine MRI. Neurology 2000;54:697–702.Article PubMed
2. Savoiardo M, Strada L, Girotti F. Olivopontocerebellar atrophy: MR diagnosis and relationship to multiple system atrophy. Radiology 1990;174:693–696.Article PubMed

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The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature
Ansuya Kasavelu Naidoo, Cait‐Lynn Deanne Wells, Yashvir Rugbeer, Neil Naidoo
Movement Disorders Clinical Practice.2022; 9(8): 1105. CrossRef



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