Autopsy-proven, sporadic pick disease with onset at age 25 years.

CONTEXT Pick disease is uncommon and accounts for less than 2% of adult-onset dementias. Reports of Pick disease in young adults have apparently increased in the last decade. OBJECTIVE To document the presentation and course of a patient with tau-positive Pick disease presenting at an extremely young age. SETTING A university hospital. PATIENT A white woman with cognitive impairment that began at age 25 years. She experienced progressive dementia over an 8-year period with radiographic evidence of severe cerebral atrophy of the frontotemporal lobes. Autopsy findings confirmed the diagnosis of Pick disease characterized by tau-positive Pick bodies in the neurons of the fascia dentata. CONCLUSION Pick disease should be considered in the differential diagnosis of young adults presenting with behavioral symptoms, especially those of frontal impairment.