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.ArticlePubMed
- 2. Savoiardo M, Strada L, Girotti F. Olivopontocerebellar atrophy: MR diagnosis and relationship to multiple system atrophy. Radiology 1990;174:693–696.ArticlePubMed
Citations
Citations to this article as recorded by
- 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