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Arch Neurol. 2007 Oct;64(10):1449-54.

TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusions.

Author information

1
Department of Neurology, University of Pennsylvania School of Medicine, 2 Gibson, 3400 Spruce St, Philadelphia, PA 19104-4283, USA. mgrossma@mail.med.upenn.edu

Abstract

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.

PMID:
17923628
DOI:
10.1001/archneur.64.10.1449
[Indexed for MEDLINE]

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