A case of early-onset rapidly progressive dementia.

A 62-year-old man presented with rapidly progressive cognitive decline associated with ataxia, spasticity, and eventually seizures. Multiple bihemispheric foci of calcification in the brain were seen on computed tomography scan, with magnetic resonance imaging (MRI) showing relatively symmetrical areas of enhancement in the brain corresponding mostly to the areas of calcification. Cerebrospinal fluid analysis and 2 brain biopsy specimens showed no evidence of an infectious, inflammatory, or malignant process. Despite being treated empirically with high-dose corticosteroids, the patient continued to deteriorate, with the family deciding for hospice care. The final autopsy diagnosis and the approach to the clinical data are discussed.