Journal
ARCHIVES OF NEUROLOGY
Volume 64, Issue 10, Pages 1449-1454Publisher
AMER MEDICAL ASSOC
DOI: 10.1001/archneur.64.10.1449
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Funding
- NIA NIH HHS [R01 AG015116, AG15116, P01 AG017586, K08 AG020073, AG17586, P01 AG019724, AG20073] Funding Source: Medline
- NINDS NIH HHS [NS44266, R01 NS044266] Funding Source: Medline
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Background: TDP-43 is a major ubiquitinated disease protein in the pathologic condition of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). Objective: To investigate the demographic, clinical, and neuropsychological features associated with subtypes of FTLD-U with TDP-43 inclusions (FTLD-U/TDP-43). Design: Retrospective clinical-pathologic study. Setting: Academic medical center. Patients: Twenty-three patients with histopathologically proven FTLD-U. Main Outcome Measures: Demographic, symptom, neuropsychological, and autopsy characteristics. Results: 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. Conclusion: Distinct TDP-43 profiles may affect clinical phenotypes differentially in patients with FTLD-U.
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