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Pick’s Disease and other Frontotemporal Dementias

Céline Chayer MD, FRCPC, Behavioural Neurology, Hôpital Maisonneuve Rosemont, Montreal, PQ.

Introduction
The prevalence of dementia in Canada has been estimated at 8%, after the age of 65 years.1 Alzheimer disease (AD) accounts for approximately 60% of cases, while other conditions accounting for the remaining 40% include, among others, vascular dementia, dementia with Lewy bodies (DLB) and frontotemporal lobar degeneration (FTLD). Differences in prognosis and symptomatic treatment, as well as eventually disease-modifying therapy underline the importance of the differential diagnosis of dementia. The use of clinical criteria for diagnosis of degenerative and vascular dementias can increase the level of clinical diagnosis accuracy2 and should, therefore, be known by physicians dealing with dementia. Advances in the understanding of FTLD have been made over the past 15 years. We will review the clinical manifestations of FTLD and highlight the differences with AD.

Pick's disease, fronto-temporal lobar degeneration and Pick complex
In 1892, Arnold Pick described patients with predominant language impairment in whom focal atrophy of the frontal and temporal lobes was found.3 Later, Alois Alzheimer described, in Pick's original patients, ballooned cortical neurons containing cytoplasmic inclusions that were subsequently called Pick bodies. Pick's disease became synonymous with frontotemporal dementia.