Abstract

A 48-year-old woman was admitted with a 3-year history of intermittent dizziness and unstable gait. Cranial MRI demonstrated a laminated lesion of T2 hyperintensity and T1 hypointensity involving the left cerebellar hemisphere, which appeared enlarged (figure, A–C). Secondary hydrocephalus and Chiari I malformation were observed. Because the striated appearance on MRI was characteristic of Lhermitte-Duclos disease,1 presurgical diagnosis was made. Total resection of this lesion was performed, and histopathologic evaluation confirmed the diagnosis (figure, D). Follow-up neuroradiologic studies revealed no recurrence. However, recent ultrasonography showed multiple nodules in the patient’s right breast, which was predictive of a confident clinical diagnosis of Cowden disease.2

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