PICKʼS DISEASE

Abstract

BACKGROUND- New findings with regard to the genetics and pathology of Pick's disease have recently been reported. These new findings are reviewed in relation to previous studies, as well as to clinical, nosological, and treatment information on Pick's disease. REVIEW SUMMARY- Pick's disease is a progressive dementia characterized by prominent personality change, cognitive deficits, elements of the Kluver-Bucy syndrome, and frontotemporal atrophy on neuroimaging. Pick's disease is suspected in the presence of a frontotemporal dementia (FTD) and frontotemporal atrophy on neuroimaging, but it cannot be diagnosed definitively without neuropathologic examination to confirm the presence of Pick bodies. Pick's disease comprises 2 to 3% of all dementias and 20 to 25% of FTDs. Age of onset averages 57 years, with a wide range (37 to 71 years), with men and women equally affected. Although commonly mistaken for Alzheimer's disease (AD), Pick's disease can be differentiated from AD by an earlier mean age of onset and prominent personality changes with relatively preserved memory and visuospatial abilities in the early stages. Clinical variants of Pick's disease include progressive aphasia, corticobasal-ganglionic degeneration, amyotrophic lateral sclerosis, and obsessive-compulsive disorder-like presentations. Symptomatic therapies, such as carbamazepine for Kluver-Bucy symptoms and clomipramine and the selective serotonin reuptake inhibitors for compulsions, can be useful. Approximately 40% of patients have a first-degree relative with a FTD and a recent linkage to chromosome 17q21–22, and tau mutations have been reported. CONCLUSIONS- Recent genetic findings may provide a much better understanding of the mechanism of atrophy in Pick's disease and elucidate its relationship to Pick complex and other FTDs.