title: 'Hot cross bun' sign in a case of cerebrotendinous xanthomatosis: a rare neuroimaging observation.
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.
authors: Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India. drrsjain@yahoo.com
authors: Rajendra Singh
authors: RS
authors: Jain
authors: Raghavendra Bakki
authors: RB
authors: Sannegowda
authors: Amit
authors: A
authors: Agrawal
authors: Deepika
authors: D
authors: Hemrajani
authors: Rahul
authors: R
authors: Jain
authors: Tarun
authors: T
authors: Mathur
mesh_terms: D000125:Achilles Tendon / Q000473:pathology*; D000328:Adult; D001706:Biopsy; D003071:Cognition; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D059906:Neuroimaging / Q000379:methods*; D011149:Pons / Q000473:pathology*; D019294:Xanthomatosis, Cerebrotendinous / Q000175:diagnosis* / Q000503:physiopathology