Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotypeReportar como inadecuado




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Acta Neuropathologica

, Volume 112, Issue 5, pp 539–549

First Online: 26 September 2006Received: 04 August 2006Revised: 24 August 2006Accepted: 24 August 2006

Abstract

We have investigated the extent and pattern of immunostaining for ubiquitin protein UBQ in 60 patients with frontotemporal lobar degeneration FTLD with ubiquitin-positive, tau-negative inclusions FTLD-U, 37 of whom were ascertained in Manchester UK and 23 in Newcastle-Upon-Tyne, UK. There were three distinct histological patterns according to the form and distribution of the UBQ pathology. Histological type 1 was present in 19 patients 32% and characterised by the presence of a moderate number, or numerous, UBQ immunoreactive neurites and intraneuronal cytoplasmic inclusions within layer II of the frontal and temporal cerebral cortex, and cytoplasmic inclusions within granule cells of the dentate gyrus; neuronal intranuclear inclusions NII of a -cat’s eye- or -lentiform- appearance were present in 17 of these patients. In histological type 2 16 patients, 27%, UBQ neurites were predominantly, or exclusively, present with few intraneuronal cytoplasmic inclusions within layer II of the cerebral cortex, while in histological type 3 25 patients, 42%, UBQ intraneuronal cytoplasmic inclusions either within the cortical layer II or in the granule cells of the dentate gyrus, with few or no UBQ neurites, were seen. In neither of these latter two groups were NII present. The influence of histological type on clinical phenotype was highly significant with type 1 histology being associated clinically with cases of frontotemporal dementia FTD or progressive non-fluent aphasia PNFA, type 2 histology with semantic dementia SD, and type 3 histology with FTD, or FTD and motor neurone disease MND.

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Autor: Ian R. A. Mackenzie - Atik Baborie - Stuart Pickering-Brown - Daniel Du Plessis - Evelyn Jaros - Robert H. Perry - David

Fuente: https://link.springer.com/







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