Format

Send to

Choose Destination
BMJ Case Rep. 2013 Feb 14;2013. pii: bcr2012006641. doi: 10.1136/bcr-2012-006641.

'Hot cross bun' sign in a case of cerebrotendinous xanthomatosis: a rare neuroimaging observation.

Author information

1
Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India. drrsjain@yahoo.com

Abstract

We report a 25-year-old young man presenting with cognitive decline, pancerebellar features, spastic quadriparesis, bilateral cataract (operated) and tendo-Achilles swelling (xanthoma). The CT of the head showed bilateral cerebellar hypodensities. There were bilateral cerebellar hypointensities involving dentate nuclei on T1-weighted images with corresponding hyperintensities on T2-weighted MRI. Additionally, an interesting MRI finding-'hot cross bun' appearance was seen in pons which has not been reported in the literature so far. Biopsy from tendo-Achilles confirmed xanthoma. He was treated with chenodeoxycholic acid following which he showed improvement in cognition and weakness.

PMID:
23417372
PMCID:
PMC3604450
DOI:
10.1136/bcr-2012-006641
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for HighWire Icon for PubMed Central
Loading ...
Support Center