Familial dementia with Pick's cells – a case report of probable familial Pick's disease

The clinical and pathological features are described in a case of prolonged dementia in a 59 year old man with familial dementia and extrapyramidal disorder. Postmortem examination showed severe fronto-temporal and basal ganglia atrophy, with many “ballooned neurons” in the residual cortex most likely representing Pick's disease. The differential diagnosis is discussed and aspects of this rare condition are reviewed.