The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia
Article first published online: 27 JAN 2009
© 2009 The Author(s). Journal compilation © 2009 EFNS
European Journal of Neurology
Volume 16, Issue 4, pages 513–516, April 2009
How to Cite
Lee, Y.-C., Liu, C.-S., Wu, H.-M., Wang, P.-S., Chang, M.-H. and Soong, B.-W. (2009), The ‘hot cross bun’ sign in the patients with spinocerebellar ataxia. European Journal of Neurology, 16: 513–516. doi: 10.1111/j.1468-1331.2008.02524.x
- Issue published online: 19 MAR 2009
- Article first published online: 27 JAN 2009
- Received 28 September 2008 Accepted 8 December 2008
- ‘hot cross bun’ sign;
- pontine midline hyperintensity;
Background and purpose: The ‘hot cross bun’ sign (HCBS), typically seen in the patients with multiple system atrophy, refers to a cruciform hyperintensity in the pons on T2-weighted MRI. Little is known about its pathological basis and prevalence in other degenerative cerebellar diseases and healthy population. We investigate the frequency of HCBS in the patients with spinocerebellar ataxia (SCA) and healthy controls.
Methods: The presence of HCBS on T2-weighted axial MRIs from 138 SCA patients (three SCA1, 35 SCA2, 76 SCA3, 18 SCA6, one SCA7, three SCA8, and two SCA17) and 102 healthy controls was evaluated retrospectively.
Results: The overall prevalence of HCBS in the SCA patients is 8.7%, but the frequency varies in different subtypes: 25.7% in SCA2, 1.3% in SCA3, and none in SCA6 or healthy controls. Notably, one patient with SCA7 and one with SCA8 were also found to have HCBS.
Conclusions: The differential list of HCBS should be expanded to include SCA7 and SCA8. The elucidation of frequency of HCBS in various SCA subtypes may help prioritize the genetic testing in late-onset dominant ataxia.