Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)
Subtle symmetrical tigrone T2-hypointensity within the pons and severe superior cerebellar vermian atrophy.
35-year-old patient presented with ataxia.
MRI showed atrophy of the superior vermis, hyperintensity in the middle cerebellar peduncles and, most characteristically, tigroid T2-hypointensity within the pons.