engleski

Expanding the neuropathological spectrum of frontotemporal lobar degenerations: review of 833 prospectively assessed dementia cases.

Background: The clinical phenotype of frontotemporal dementia (FTD) encompasses two progressive patterns: gradual behavioral change and language disorder. Collectively, FTD may be caused by a diverse group of neurodegenerative diseases called frontotemporal lobar degenerations (FTLDs). New entities have been described and the nosology of FTLDs continues to evolve. Objective: To determine the type and frequency of FTLDs in a series using contemporary immunohistochemical methods. Methods: Eight hundred and thirty-three dementia cases were prospectively assessed at Washington University Alzheimer’ s Disease Research Center (WUADRC) and cases with clinical FTD were identified using existing diagnostic criteria and neuropathologic entities were ascertained using immunohistochemistry and contemporary diagnostic criteria. Results: Of the dementia cases, 53(6.3%) met clinical criteria for FTD ; 45(5.1%) fulfilled both clinical and neuropathological criteria for FTLD, and another 8 fulfilled only the clinical criteria. Forty percent of the cases were tauopathies: Pick’ s disease(n=3) ; corticobasal degeneration(n=11), argyrophilic grain disease(AGD, n=2), frontotemporal dementia with parkinsonism linked to chromosome 17(n=2), neurofibrillary tangle dementia(n=2), and one familial tauopathy with no tau mutation(n=1). However, most FTLD cases were characterized by ubiquitin-positive, tau-negative inclusions: FTLD with motor neuron disease-type inclusions (FTLD-MND-type, n=25, 47.2%), inclusion body myositis with Paget disease and frontotemporal dementia(IBMPFD, n=1), and diffuse leukoencephalopathy with neuroaxonal spheroids (DLN, n=3). Co-existing hippocampal sclerosis and AD-type changes were observed in 12 and 10 cases, respectively. Conclusion: Although FTLD-MND-type is the most frequent FTLD in this prospectively assessed series, less common entities not included in the McKhann criteria, such as AGD, DLS, and IBMPFD, may also present clinically as FTD and should be considered as part of the neuropathologic spectrum of FTLDs that clinic.

Frontotemporal dementia; Frontotemporal lobar degeneration

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