from Part VI - Other Dementias
Published online by Cambridge University Press: 04 August 2010
Introduction
Frontotemporal lobar degeneration, formerly called Pick's disease, is a progressive dementia that is associated with focal atrophy of the frontal and/or temporal lobes. For over 100 years, the key clinical and pathological feature of this disease has been recognized to be focal, often asymmetric cortical involvement. Histopathologically, frontotemporal lobar degeneration (FTLD) is distinct from Alzheimer's disease but heterogeneous, even among similar clinical syndromes. Recently, with the advent of specialized immunohistochemical stains and insights gained from molecular genetics, it has been recognized that FTLD is closely related to, and sometimes overlaps with three other neurodegenerative diseases: corticobasal ganglionic degeneration (CBD), progressive supranuclear palsy (PSP), and motor neuron disease (MND). The central role of the microtubule-associated protein, tau, in the pathogenesis of FTLD, has led to classification as a “tauopathy.”
First description and history of FTLD
The first case of what is now called frontotemporal lobar degeneration (FTLD) was described by Arnold Pick in 1892 (Pick, 1892). His subsequent description of six similar patients emphasized a language impairment, which he termed “amnestic aphasia,” and a focal pattern of brain atrophy involving the temporal and/or frontal lobes. A lack of senile plaques and tangles in the brains of similar patients was noted by Alzheimer in 1911. He and Altman provided the first histopathological description of argyrophilic inclusions (later termed Pick bodies) and swollen achromatic cells (later termed Pick cells) (Altman, 1923).
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