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Titolo:
Late-onset neurodegeneration with brain iron accumulation type 1: Expanding the clinical spectrum
Autore:
Racette, BA; Perry, A; DAvossa, G; Perlmutter, JS;
Indirizzi:
Washington Univ, Sch Med, Dept Neurol & Neurol Surg, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 Neurol Surg, St Louis, MO 63110 USA Washington Univ, Sch Med, Edward Mallinckrodt Inst Radiol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 Inst Radiol, St Louis, MO 63110 USA Washington Univ, Sch Med, Dept Pathol Neuropathol, St Louis, MO USA Washington Univ St Louis MO USA ept Pathol Neuropathol, St Louis, MO USA Washington Univ, Sch Med, Dept Anat & Neurobiol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 & Neurobiol, St Louis, MO 63110 USA
Titolo Testata:
MOVEMENT DISORDERS
fascicolo: 6, volume: 16, anno: 2001,
pagine: 1148 - 1152
SICI:
0885-3185(200111)16:6<1148:LNWBIA>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
HALLERVORDEN-SPATZ-SYNDROME; BASAL GANGLIA; HEMIBALLISMUS; PARKINSONISM; HEMICHOREA; LESIONS; DISEASE;
Keywords:
Hallervorden-Spatz; parkinsonism; hemiballismus; dementia;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
21
Recensione:
Indirizzi per estratti:
Indirizzo: Racette, BA Washington Univ, Sch Med, Dept Neurol & Neurol Surg, 660 S Euclid Ave,Box 8111, St Louis, MO 63110 USA Washington Univ 660 S Euclid Ave,Box 8111 St Louis MO USA 63110
Citazione:
B.A. Racette et al., "Late-onset neurodegeneration with brain iron accumulation type 1: Expanding the clinical spectrum", MOVEMENT D, 16(6), 2001, pp. 1148-1152

Abstract

We report on two patients with pathologically proven neurodegeneration with brain iron accumulation type 1 (NBIA-1) with late onset and atypical presentations. One patient experienced gradual onset of shuffling gait, rigidity, bradykinesia, and increasing postural instability at age 85 years. He died a few weeks after developing acute hemiballismus at age 90 years. Histopathology revealed marked neuronal loss in the internal segment of the globus pallidum, astrocytosis, axonal spheroids, and extensive iron deposition consistent with NBIA-1. No additional lesions were found to explain the hemiballismus. The second patient experienced fulminant dementia evolving to total disability and death within 2 months. Autopsy showed typical NABIA-1 pathology. We conclude that NBIA-1 pathology can develop at any age, and thatthe phenotype should be expanded to include late-onset parkinsonism. The relationship to hemiballismus and adult-onset dementia is less clear. (C) 2001 Movement Disorder Society.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/06/19 alle ore 17:57:23