Fig. 1.

MRI results for the proband. (A, B): T1 sagittal and T2 transversal view in 2008 at onset of MSA symptoms. Only mild cerebellar atrophy was noted. (C, D): sagittal and transversal views 3 years later. Cerebellar atrophy (blue arrow) had progressed and additional brainstem atrophy with the typical “hot-cross bun sign” had appeared (yellow arrow). E. Pedigree demonstrating prion and MSA diseases in the family. The prion-affected cases are represented in yellow; the MSA-affected cases are represented in blue. Abbreviation: MSA, multiple system atrophy.