Journal Article

The evolution and pathology of frontotemporal dementia

Andrew Kertesz, Paul McMonagle, Mervin Blair, Wilda Davidson and David G. Munoz

in Brain

Published on behalf of The Guarantors of Brain

Volume 128, issue 9, pages 1996-2005
Published in print September 2005 | ISSN: 0006-8950
Published online July 2005 | e-ISSN: 1460-2156 | DOI:
The evolution and pathology of frontotemporal dementia

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This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.

Keywords: frontotemporal dementia; primary progressive aphasia; corticobasal degeneration; progressive supranuclear palsy; Pick's disease; CBD = corticobasal degeneration (pathology); CBDS = corticobasal degeneration syndrome (clinical); DLDH = dementia lacking distinctive histology; FTD = frontotemporal dementia; FTD-bv = behavioural variant of frontotemporal dementia; FTLD = frontotemporal lobar degeneration; MND = motor neuron disease; MNDI = motor neuron disease type inclusions; PA = progressive aphasia (secondary); PPA = primary progressive aphasia; PSP = progressive supranuclear palsy; SD = semantic dementia

Journal Article.  6094 words.  Illustrated.

Subjects: Neurology ; Neuroscience

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