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Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)

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  • Subtle symmetrical tigrone T2-hypointensity within the pons and severe superior cerebellar vermian atrophy.

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  • 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.