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Frontotemporal Lobar Degeneration With Motor Neuron Disease
A Clinical and Pathological Spectrum
Arch Neurol. 2006;63:489-490.
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Frontotemporal lobar degeneration (FTLD) is second only to Alzheimer disease as a cause of progressive cognitive impairment. The diagnostic category encompasses a phenotypic and pathologically heterogeneous group of disorders that begs to be split into distinct, clinically recognizable units. The clinical phenotype involves a variable spectrum of impairments in behavior and language, psychiatric symptoms, and motor signs in pyramidal and extrapyramidal systems.1-3
Based on the clinical features, patients are grouped into behavioral (frontotemporal dementia) and language (primary progressive aphasia and semantic dementia) subtypes or into specific clinical syndromes such as frontotemporal dementia with motor neuron disease (FTD-MND), corticobasal degeneration, and progressive supranuclear palsy. The pathologic spectrum associated with FTLD is equally heterogeneous, with the initial division based on immunohistochemical evidence for the presence of cytoplasmic aggregation of the microtubule associated protein tau.1-2 Those with tau inclusions (ie, tauopathies) are further categorized based on distinctive histopathologic and biochemical findings into Pick . . . [Full Text of this Article] AUTHOR INFORMATION
Christopher M. Clark, MD;
Mark S. Forman, MD, PhD
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