The evolution and pathology of frontotemporal dementia

Abstract

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.

[18F]AV‐1451 PET in behavioral variant frontotemporal dementia due to MAPT mutation

“…The patient had particularly abnormal BP ND in anterior temporal lobes and ventral anterior cingulate cortex; areas that are particularly prone to tau accumulation in frontotemporal dementia,20 and that were neuropathologically most abnormal in her father.…”

L'aphasie (primaire) progressive: un diagnostic simple ou complexe

“…Néanmoins, les nombreuses études de cas et surtout les suivis longitudinaux avec confirmation anatomopathologique des AP au sens large ont montré une grande hétérogénéité des tableaux neurolinguistiques et d'importants recouvrements avec d'autres maladies neurodégénératives comme la paralysie supranucléaire progressive (PSP), la dégénérescence corticobasale (DCB), la maladie du motoneurone (MMN), ou encore la démence frontotemporale (DFT) [10][11][12][13]. Ces données, en particulier celles avec suivi longitudinal et autopsie ont révélé que le syndrome langagier peut non seulement précéder, mais également suivre un syndrome neurologique ou neuropsy- …”

Papel da proteína Tau na fisiopatologia da demência frontotemporal

“…Em 1892, Arnold Pick descreveu casos clínicos que cursavam com deterioração cognitiva, afasia progressiva e mudanças de conduta social, sendo essas manifestações associadas com a atrofia dos lobos temporais e frontais 1 . Um século depois, os grupos de pesquisa de Lund, na Suécia, e Manchester, na Inglaterra, publicaram crité-rios clínicos e neuropatológicos para o diagnóstico da demência frontotemporal (DFT), que ainda levam em consideração a presença e a distribuição das células (ou corpúsculos) de Pick 2 .…”

“…Outros marcadores, como perdas sinápticas e neuronais nas áreas afetadas podem ter mais correlação com o comprometimento motor e cognitivo 11 . A patologia da DFT pode ser dividida em duas variáveis: Tau positiva ou Tau negativa 1 . O objetivo deste estudo é rever o papel da proteína Tau na patogenia da DFT.…”