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Grossman M, Wood EM, Moore P, et al. TDP-43 Pathologic Lesions and Clinical Phenotype in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions. Arch Neurol. 2007;64(10):1449–1454. doi:https://doi.org/10.1001/archneur.64.10.1449
TDP-43 is a major ubiquitinated disease protein in the pathologic condition of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U).
To investigate the demographic, clinical, and neuropsychological features associated with subtypes of FTLD-U with TDP-43 inclusions (FTLD-U/TDP-43).
Retrospective clinical-pathologic study.
Academic medical center.
Twenty-three patients with histopathologically proven FTLD-U.
Main Outcome Measures
Demographic, symptom, neuropsychological, and autopsy characteristics.
There are notably different clinical and neuropsychological patterns of impairment in FTLD-U subtypes. Patients with FTLD-U/TDP-43 characterized by numerous neuronal intracytoplasmic inclusions have shorter survival; patients with FTLD-U/TDP-43 featuring numerous neurites have difficulty with object naming; and patients with FTLD-U/TDP-43 in whom neuronal intranuclear inclusions are present have substantial executive deficits. There are also different anatomical distributions of ubiquitin pathologic features in FTLD-U subgroups, consistent with their cognitive deficits.
Distinct TDP-43 profiles may affect clinical phenotypes differentially in patients with FTLD-U.
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