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Multiple system atrophy (MSA) typically presents with parkinsonism, cerebellar ataxia, corticospinal dysfunction, and autonomic failure.1 We describe a case of pathologically confirmed MSA with semantic language impairment.
Case report.
A 55-year-old right-handed psychiatrist presented with language difficulties since age 50. He had shrinking vocabulary, spelling, and reading difficulties, loss of foreign language skills, and difficulty with comprehension of complex conversations. His speech was fluent, grammatically correct, with occasional anomic pauses and substitution of low-frequency words with general words (e.g., “thing” and “it”). He had hyperreflexia in the right arm and leg, right-sided Babinski, writer's cramp, and mildly impaired fine motor skills in the right hand. Neuropsychological evaluation revealed anomia (Boston Naming Test [BNT] score 35/60, <1%), intact verbal fluency (FAS 88%), surface dysgraphia (“curteus” instead of “courteous,” “medeval” instead of “medieval”), surface dyslexia (could not read “subtlety”), and moderate retrieval-type verbal memory deficit. His receptive vocabulary skills were below expectation given his advanced education (Peabody Picture Vocabulary Test, 48%). His performance …
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