| Clinical features and survival of 3R and 4R tauopathies presenting as behavioral variant frontotemporal dementia. | |
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MedLine Citation:
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PMID: 18090422 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We compared the clinical characteristics of 3 repeat (3R) and 4 repeat (4R) tau-positive cases (tauopathies) presenting as behavior variant frontotemporal dementia (bv-FTD). We identified and retrospectively reviewed demographics and clinical features of patients with pathologically confirmed tau-positive frontotemporal lobar degeneration in a blinded fashion. Those presenting as bv-FTD were divided according to their tau isoform, 3R versus 4R, and compared with age-matched and sex-matched control patients with 4R tauopathies but presenting clinical syndromes other than bv-FTD. Twenty-four cases with tau-positive bv-FTD and 18 4R tau-positive controls were included in the study. Patients with 4R tauopathies had significantly shorter disease duration than patients with 3R tauopathy (median, 6.5 y vs. 9.5 y; P<0.05), despite similar age of disease onset and regardless of whether bv-FTD was the presenting clinical syndrome. Among bv-FTD cases, those with 4R tauopathies were more likely to display behavioral underactivity than those with 3R tauopathy (P=0.03), although 3R and 4R tauopathy patients shared many similar clinical features. In summary, survival in 4R tauopathies seemed independent of the presenting clinical phenotype, and there may be subtle clinical differences between bv-FTD patients with 3R and 4R tauopathies. |
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Authors:
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William T Hu; Joseph E Parisi; David S Knopman; Bradley F Boeve; Dennis W Dickson; J Eric Ahlskog; Ronald C Petersen; Keith A Josephs |
Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Alzheimer disease and associated disorders Volume: 21 ISSN: 0893-0341 ISO Abbreviation: Alzheimer Dis Assoc Disord Publication Date: 2007 Oct-Dec |
Date Detail:
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Created Date: 2007-12-19 Completed Date: 2008-02-28 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8704771 Medline TA: Alzheimer Dis Assoc Disord Country: United States |
Other Details:
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Languages: eng Pagination: S39-43 Citation Subset: IM |
Affiliation:
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Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Dementia
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physiopathology* Diagnosis, Differential Humans Protein Isoforms Tauopathies / mortality*, physiopathology* tau Proteins* |
| Grant Support | |
ID/Acronym/Agency:
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HD49078/HD/NICHD NIH HHS; P50 AG16574/AG/NIA NIH HHS; U01 AG06786/AG/NIA NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Protein Isoforms; 0/tau Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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