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Titolo:
Progress in clinical neurosciences: The evidence for ALS as a multisystemsdisorder of limited phenotypic expression
Autore:
Strong, MJ;
Indirizzi:
Univ Western Ontario, Dept Clin Neurol Sci, London, ON, Canada Univ Western Ontario London ON Canada lin Neurol Sci, London, ON, Canada
Titolo Testata:
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
fascicolo: 4, volume: 28, anno: 2001,
pagine: 283 - 298
SICI:
0317-1671(200111)28:4<283:PICNTE>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; MOTOR-NEURON DISEASE; PLACEBO-CONTROLLED TRIAL; GLUTATHIONE-PEROXIDASE ACTIVITIES; SUPEROXIDE-DISMUTASE MUTATION; POSITRON EMISSION TOMOGRAPHY; CEREBRAL BLOOD-FLOW; GROWTH-FACTOR-I; THYROTROPIN-RELEASING-HORMONE; HEAVY NEUROFILAMENT SUBUNIT;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
222
Recensione:
Indirizzi per estratti:
Indirizzo: Strong, MJ London Hlth Sci Ctr, Room 7OF10,Univ Campus,339 Windermere Rd, London, ON N6A 5A5, Canada London Hlth Sci Ctr Room 7OF10,Univ Campus,339 Windermere Rd London ON Canada N6A 5A5
Citazione:
M.J. Strong, "Progress in clinical neurosciences: The evidence for ALS as a multisystemsdisorder of limited phenotypic expression", CAN J NEUR, 28(4), 2001, pp. 283-298

Abstract

Traditionally, amyotrophic lateral sclerosis (ALS) is considered to be a unique neurodegeneration disorder in which motor neurons are selectively vulnerable to a single disease process. Our current understanding of ALS, however, suggests that this is far too limited an approach. While motor neuron degeneration remains the central component to this process, there is considerable phenotypic variability including broad ranges in survivorship and the presence or absence of cognitive impairment. The number of familial variants of ALS for which unique genetic linkage has been identified is increasing, attesting further to the biological heterogeneity of the disorder. At the cellular level, derangements in cytoskeletal protein and glutamate metabolism, mitochondrial function, and in glial interactions are clearly evident. When considered in this fashion, ALS can be justifiably considered a disorder of multiple biological processes sharing in common the degeneration of motor neurons.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 24/09/20 alle ore 08:22:49