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Bilateral hyperintense lesions in cerebrotendinous xanthomatosis (MRI)

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This axial T2-weighted MRI of a 42-year-old man with cerebrotendinous xanthomatosis shows bilateral hyperintense lesions involving the dentate nuclei and deep cerebellar white matter. Clinical presentation: He had a history of early-onset cataracts, cognitive difficulty, and gait instability. Examination revealed nasal speech, severe dysarthria, marked ataxia of limbs and gait, and bilateral Achilles tendon xanthomas. (Source: Islam M, Hoggard N, Hadjivassiliou M. Cerebrotendinous xanthomatosis: diversity of presentation and refining treatment with chenodeoxycholic acid. Cerebellum Ataxias 2021;8[1]:5. Creative Commons Attribution 4.0 International License.)