Pick's Disease

History

The initial criteria developed to recognize FTD was the Lund and Manchester Groups (Lund–Manchester) criteria published in 1994 that required the presence of at least two of the following features: loss of personal awareness, strange eating habits, perseveration, and mood change. In addition, patients needed to have at least one of the following: frontal executive dysfunction, reduced speech, and preserved visuospatial ability. Supportive features included onset before 65 years of age, a family history of FTD, early urinary incontinence, motor neuron disease, and (during the late stages) akinesia, rigidity, and tremor.

David Neary and colleagues in 1998 developed the second set of consensus guidelines for diagnosing FTD, retaining the general framework of the Lund–Manchester criteria but subdividing the patients into three distinct clinical presentations: frontotemporal dementia (personality change and social conduct disturbance), progressive nonfluent aphasia, and semantic [Page 464]aphasia. Frontotemporal lobar degeneration was the term used to refer to any of the three symptoms.

The diagnostic framework was further refined in 2001 by the Work Group on Frontotemporal Dementia and Pick's Disease, which eliminated the three subdivisions in favor of calling all of them frontotemporal dementia but noted that patients would experience either a behavioral presentation or a language presentation to their illness. Specific criteria included the development of behavioral or cognitive deficits manifested by either (a) early and progressive change in personality, characterized by difficulty in modulating behavior, often resulting in inappropriate responses or activities, or (b) early and progressive change in language, characterized by problems with expression of language or severe naming difficulty and problems with word meaning. These deficits must cause significant impairment in social or occupational functioning and represent a decline from a previous level of functioning and must not be better accounted for by another medical condition, delirium, or psychiatric condition.

Clinical Presentation

Pick's disease differs from Alzheimer's disease in several ways, and the two diseases are distinct in their neuropathology. Pick's disease is identified by the presence of Pick bodies; that is, rounded structures identified within affected neurons that give them a balloon-like appearance. The characteristic plaques and tangles found in Alzheimer's disease are not typically present in Pick's disease, and the two types of dementia affect primarily different cerebral structures. Pick's disease is characterized by significant and marked frontal atrophy, whereas Alzheimer's disease appears to involve temporal structures early and then progresses to involve parietal areas and frontal areas later in the course of the disease. This differing neuropathological distribution corresponds to the clinical course of the two diseases, as the early signs of Pick's disease commonly involve personality changes, whereas the early signs of Alzheimer's disease involve anterograde memory.

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