Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
A clinical and pathological study of motor neurone disease on Guam
Autore:
Morris, HR; Al-Sarraj, S; Schwab, C; Gwinn-Hardy, K; Perez-Tur, J; Wood, NW; Hardy, J; Lees, AJ; McGeer, PL; Daniel, SE; Steele, JC;
Indirizzi:
Guam Mem Hosp, Tamuning, GU 96911 USA Guam Mem Hosp Tamuning GU USA 96911Guam Mem Hosp, Tamuning, GU 96911 USA Univ Coll London, Univ Dept Clin Neurol, Neurogenet Sect, London, England Univ Coll London London England eurol, Neurogenet Sect, London, England Univ Coll London, Inst Neurol, Parkinsons Dis Soc Brain Res Ctr, London, England Univ Coll London London England Dis Soc Brain Res Ctr, London, England Univ Coll London, Natl Hosp Neurol & Neurosurg, London, England Univ Coll London London England osp Neurol & Neurosurg, London, England Univ Coll London, Inst Psychiat, Dept Neuropathol, London, England Univ Coll London London England hiat, Dept Neuropathol, London, England Univ Coll London, Reta Lila Weston Inst Neurol Sci, London, England Univ Coll London London England Weston Inst Neurol Sci, London, England Inst Biomed, Unitat Genet Mol, Valencia, Spain Inst Biomed Valencia Spain st Biomed, Unitat Genet Mol, Valencia, Spain Mayo Clin, Neurogenet Lab, Jacksonville, FL 32224 USA Mayo Clin Jacksonville FL USA 32224 genet Lab, Jacksonville, FL 32224 USA Univ British Columbia, Kinsmen Lab Neurol Res, Vancouver, BC V5Z 1M9, Canada Univ British Columbia Vancouver BC Canada V5Z 1M9 ver, BC V5Z 1M9, Canada
Titolo Testata:
BRAIN
, volume: 124, anno: 2001,
parte:, 11
pagine: 2215 - 2222
SICI:
0006-8950(200111)124:<2215:ACAPSO>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; PARKINSONISM-DEMENTIA COMPLEX; SUPEROXIDE-DISMUTASE GENE; NEUROFIBRILLARY DEGENERATION; TAU; TANGLES; ALS;
Keywords:
Guam amyotrophic lateral sclerosis; motor neurone disease; neurofibrillary tangles; parkinsonism-dementia complex; pathology;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
34
Recensione:
Indirizzi per estratti:
Indirizzo: Steele, JC Guam Mem Hosp, Tamuning, GU 96911 USA Guam Mem Hosp Tamuning GU USA 96911 sp, Tamuning, GU 96911 USA
Citazione:
H.R. Morris et al., "A clinical and pathological study of motor neurone disease on Guam", BRAIN, 124, 2001, pp. 2215-2222

Abstract

Despite over 40 years of intensive study, the cause of the high incidence of motor neurone disease (MND) on Guam, and the relationship between this disease and MND seen in the rest of the world are still uncertain. We present a series of 45 cases of Guamanian MND, which reaffirm the clinical similarity between this disease and MND seen in other countries. However, the occurrence of MND among the indigenous Chamorros of Guam is distinguished by four factors: (i) high prevalence; (ii) frequent familial occurrence; (iii) co-occurrence with the parkinsonism-dementia complex (PDC); and (iv) association with an unusual and distinctive linear retinopathy termed Guam retinal pigment epitheliopathy (GRPE). These distinguishing factors were not present in four non-Chamorros who resided on Guam when their MND symptoms occurred. Pathologically, the classical features of MND were seen in Guamanian Chamorro cases including ubiquitin inclusions. Neurofibrillary tangles were frequently seen. The neurofibrillary tangles appeared in the same distribution as described in the PDC but, unlike classical PDC, they were not usually associated with cell loss and occurred less frequently. While neurofibrillary tangle formation and the clinicopathological syndrome of MND may occurin parallel, observations from this series suggest that pathologically classical MND on Guam may occur independently of neurofibrillary degeneration and the clinical features of PDC.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/11/20 alle ore 04:53:47