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P2‐189: Longitudinal atrophy rate as a biomarker of disease progression in frontotemporal lobar degeneration
Author(s) -
McNaught Elizabeth,
Rohrer Jonathan D.,
Omar Rohani,
Ahsan Laila,
Rossor Martin N.,
Warren Jason D.,
Fox Nick C.
Publication year - 2008
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2008.05.1263
Subject(s) - atrophy , frontotemporal lobar degeneration , medicine , biomarker , dementia , frontotemporal dementia , semantic dementia , cardiology , pathology , cohort , disease , biochemistry , chemistry
characterized by severe progressive psychobehavioural abnormalities in the absence of significant aphasia, cognitive-intellectual dysfunction or motor features. The neuropathological features were highly consistent, with small, round, neuronal cytoplasmic inclusions that were immunoreactive for ubiquitin (ub-ir), but negative for tau, -synuclein, intermediate filaments and TDP-43. Cytoplasmic inclusions were most numerous in the neocortex, dentate granule cells and hippocampal pyramidal neurons. Ub-ir neuronal intranuclear inclusions were also present in neocortical and hippocampal neurons and had the unusual appearance of straight, curved, or twisted filaments. Conclusions: We believe that these cases represent a new entity that is clinically and pathologically distinct from all currently recognized subtypes of FTLD. Moreover, the existence of such cases indicates that the designations of “FTLD-U” and “TDP-43 proteinopathy” should not be considered to be synonymous.