engleski

Ubiquitin-positive inclusions predominate in 48 cases of frontotemporal lobar degeneration.

Backgroud: The frontotemporal lobar degenerations (FTLDs) are clinically and neuropathologically heterogeneous and may account for 5-10% of dementias. Advances in immunohistochemistry, biochemistry, and genetics of FTLDs have helped to define this pathological spectrum of FTLDs. Aims: To determine the pathological spectrum and frequency of FTLDs in a cohort of individuals from Washington University Alzheimers Disease Research Center (WUADRC). Methods: Review of 833 prospectively assesses dementia cases between 1988 and 2005 at the WUADRC and selection of cases with a neuropathological diagnosis of FTLD according to established and other neuropathological criteria. Brain tissue was processed and blocks taken according to the established protocols of the WUADRC Neuropathology Core. Immunohistochemistry was performed on selected areas against the following peptides or proteins: ß-amyloid, phosphorylated tau, α -synuclein, ubiquitin, α -internexin, and valosin-containing protein. Results: Of 48 FTLD cases (5.7% of 833 cases), 14 were classified as containing protein tau: Pick’ s disease (n=2), corticobasal degeneration (n=9), argyrophilic grain disease (AGD, n=1), and frontotemporal dementia with parkinsonism linked to chromosome 17 (n=2). However, the majority of cases were characterized by tau-negative inclusions: FTLD with ubiquitin inclusions (FTLD-U) (n=24). One case had valosin-containing protein inclusions, also called inclusion body myositis with Paget disease and frontotemporal dementia (IBMPFD) ; or none, also called dementia lacking distinctive histopathology (DLDH, n=9). Conclusion: FTLD-U is the most frequent FTLD in this series (50%). Less common entities not typically included such as IBMPFD, AGD may also have the clinical phenotype of FTLD and should be considered as part of the neuropathological spectrum of FTLDs.

Frontotemporal lobar degeneration; Ubiquitin

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