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Titolo:
Frontal lobe dementia with novel tauopathy: Sporadic multiple system tauopathy with dementia
Autore:
Bigio, EH; Lipton, AM; Yen, SH; Hutton, ML; Baker, M; Nacharaju, P; White, CL; Davies, P; Lin, WL; Dickson, DW;
Indirizzi:
Univ Texas, SW Med Sch, Dept Pathol, Neuropathol Lab, Dallas, TX 75390 USAUniv Texas Dallas TX USA 75390 hol, Neuropathol Lab, Dallas, TX 75390 USA Univ Texas, SW Med Sch, Dept Neurol, Dallas, TX 75390 USA Univ Texas Dallas TX USA 75390 Med Sch, Dept Neurol, Dallas, TX 75390 USA Mayo Clin Jacksonville, Dept Pharmacol, Jacksonville, FL 32224 USA Mayo Clin Jacksonville Jacksonville FL USA 32224 cksonville, FL 32224 USA Mayo Clin Jacksonville, Dept Neurogenet, Jacksonville, FL 32224 USA Mayo Clin Jacksonville Jacksonville FL USA 32224 cksonville, FL 32224 USA Albert Einstein Coll Med, Dept Pathol, Bronx, NY USA Albert Einstein Coll Med Bronx NY USA ll Med, Dept Pathol, Bronx, NY USA
Titolo Testata:
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
fascicolo: 4, volume: 60, anno: 2001,
pagine: 328 - 341
SICI:
0022-3069(200104)60:4<328:FLDWNT>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROGRESSIVE SUPRANUCLEAR PALSY; FAMILIAL PRESENILE-DEMENTIA; HEREDITARY FRONTOTEMPORAL DEMENTIA; TAU-PROTEIN PATHOLOGY; CORTICOBASAL DEGENERATION; ALZHEIMERS-DISEASE; NEURODEGENERATIVE DISEASES; FTDP-17 MUTATIONS; NEUROFIBRILLARY DEGENERATION; CHROMOSOME 17Q21-22;
Keywords:
corticobasal ganglionic degeneration; dementia; frontal lobe dementia; frontotemporal degeneration; FTDP-17; progressive supranuclear palsy; tauopathy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
73
Recensione:
Indirizzi per estratti:
Indirizzo: Bigio, EH Univ Texas, SW Med Sch, Dept Pathol, Neuropathol Lab, 5323 HarryHines Blvd, Dallas, TX 75390 USA Univ Texas 5323 Harry Hines Blvd Dallas TX USA 75390 X 75390 USA
Citazione:
E.H. Bigio et al., "Frontal lobe dementia with novel tauopathy: Sporadic multiple system tauopathy with dementia", J NE EXP NE, 60(4), 2001, pp. 328-341

Abstract

We present a novel tauopathy in a patient with a 10-yr history of progressive frontal lobe dementia and a negative family history. Autopsy revealed mild atrophy of frontal and parietal lobes and severe atrophy of the temporal lobes. There were occasional filamentous tau-positive inclusions, but more interesting were numerous distinctive globular neuronal and glial tau-positive inclusions in both gray and white matter of the neocortex. Affected subcortical regions included substantia nigra, globus pallidus, subthalamic nucleus, and cerebellar dentate nucleus, in a distribution similar to progressive supranuclear palsy (PSP), but without significant accompanying neuronal loss or gliosis. Predominantly straight filaments were detected by electron microscopy (EM), while other inclusions were similar to fingerprint bodies. No twisted ribbons were detected. Immuno-EM studies revealed that only the filamentous inclusions were composed of tau. Immunoblotting of sarkosyl-insoluble tau revealed 2 major bands of 64 and 68 kDa. Blotting analysisafter dephosphorylation revealed predominantly 4-repeat tau. Sequence analysis of tau revealed that there were no mutations in either exons 9-13 or the adjacent intronic sequences. The unique cortical tau pathology in this case of sporadic multiple system tauopathy with dementia adds a new pathologic profile to the spectrum of tauopathies.

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Documento generato il 04/04/20 alle ore 01:10:50