Cruciform Pontine MRI Hyperintensities (“Hot Cross Bun” Sign) in Non-Multiple System Atrophy Patients

Article information

JMD. 2008;1(2):107-108
Publication date (electronic) : 2008 October 30
doi : https://doi.org/10.14802/jmd.08022
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 2007 October 24; Accepted 2008 May 19.

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.
2. Savoiardo M, Strada L, Girotti F. Olivopontocerebellar atrophy: MR diagnosis and relationship to multiple system atrophy. Radiology 1990;174:693–696.

Article information Continued

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.