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.