Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Clinical markers of early disease in persons near onset of Huntington's disease
Autore:
Paulsen, JS; Zhao, H; Stout, JC; Brinkman, RR; Guttman, M; Ross, CA; Como, P; Manning, C; Hayden, MR; Shoulson, I;
Indirizzi:
Univ Iowa, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242Univ Iowa, Iowa City, IA 52242 USA Univ Rochester, Rochester, NY 14627 USA Univ Rochester Rochester NY USA 14627 Rochester, Rochester, NY 14627 USA Indiana Univ, Bloomington, IN 47405 USA Indiana Univ Bloomington IN USA 47405 ana Univ, Bloomington, IN 47405 USA Univ British Columbia, Vancouver, BC V5Z 1M9, Canada Univ British Columbia Vancouver BC Canada V5Z 1M9 ver, BC V5Z 1M9, Canada Univ Toronto, Toronto, ON, Canada Univ Toronto Toronto ON CanadaUniv Toronto, Toronto, ON, Canada Johns Hopkins Univ, Baltimore, MD 21218 USA Johns Hopkins Univ Baltimore MD USA 21218 s Univ, Baltimore, MD 21218 USA Univ Virginia, Charlottesville, VA 22903 USA Univ Virginia Charlottesville VA USA 22903 Charlottesville, VA 22903 USA
Titolo Testata:
NEUROLOGY
fascicolo: 4, volume: 57, anno: 2001,
pagine: 658 - 662
SICI:
0028-3878(20010828)57:4<658:CMOEDI>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
REPEAT NEURODEGENERATIVE DISEASES; CEREBRAL GLUCOSE-METABOLISM; ASYMPTOMATIC GENE CARRIERS; COGNITIVE PERFORMANCE; PATHOGENIC MECHANISM; RECEPTOR-BINDING; AT-RISK; MANIFESTATIONS; MUTATION; MODELS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
42
Recensione:
Indirizzi per estratti:
Indirizzo: Paulsen, JS Univ Iowa, 1-289 MEB, Iowa City, IA 52242 USA Univ Iowa 1-289 MEB Iowa City IA USA 52242 City, IA 52242 USA
Citazione:
J.S. Paulsen et al., "Clinical markers of early disease in persons near onset of Huntington's disease", NEUROLOGY, 57(4), 2001, pp. 658-662

Abstract

Objective: There is increasing evidence that neuron loss precedes the phenotypic expression of Huntington's disease (HD). As genes for late-onset neurodegenerative diseases are identified, the need for accurate assessment ofphenoconversion (i.e., the transition from health to the disease phenotype) will be important. Methods: Prospective longitudinal evaluation using theUnified Huntington's Disease Rating Scale (UHDRS) was conducted by Huntington Study Group members from 36 sites. There were 260 persons considered "at risk" for HD who initially did not have manifest disease and had at leastone subsequent evaluation. Repeat UHDRS data, obtained an average of 2 years later, showed that 70 persons were given a diagnosis of definite HD based on the quantified neurologic examination. Results: Baseline cognitive performances were consistently worse for the at-risk group who demonstrated conversion to a definitive diagnosis compared with those who did not. Longitudinal change scores showed that the at-risk group who did not demonstrate manifest disease during the follow-up study period demonstrated improvementsin all cognitive tests, whereas performances in the at-risk group demonstrating conversion to disease during the study declined across cognitive domains. Conclusions: Neuropsychological measures show impairment 2 years before the development of more manifest motor disease. Findings suggest that these brief cognitive measures administered over time may capture early striatal neural loss in HD.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 12:56:45