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Titolo:
The physiological basis of conduction slowing in ALS patients homozygous for the D90A CuZn-SOD mutation
Autore:
Weber, M; Eisen, A; Stewart, HG; Andersen, PM; Hirota, N;
Indirizzi:
Vancouver Gen Hosp, Neuromuscular Dis Unit, Vancouver, BC V5Z 1M9, Canada Vancouver Gen Hosp Vancouver BC Canada V5Z 1M9 couver, BC V5Z 1M9, Canada Univ British Columbia, Vancouver, BC V5Z 1M9, Canada Univ British Columbia Vancouver BC Canada V5Z 1M9 ver, BC V5Z 1M9, Canada Umea Univ, Inst Clin Neurosci, Umea, Sweden Umea Univ Umea SwedenUmea Univ, Inst Clin Neurosci, Umea, Sweden
Titolo Testata:
MUSCLE & NERVE
fascicolo: 1, volume: 24, anno: 2001,
pagine: 89 - 97
SICI:
0148-639X(200101)24:1<89:TPBOCS>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYOTROPHIC-LATERAL-SCLEROSIS; MAGNETIC BRAIN-STIMULATION; MOTOR-NEURON DISEASE; CU/ZN SUPEROXIDE-DISMUTASE; MULTIPLE-SCLEROSIS; CORTICOSPINAL PROJECTIONS; SPINAL MOTONEURONS; EVOKED-POTENTIALS; CORTICOMOTONEURONAL PSTH; CORTICAL EXCITABILITY;
Keywords:
amyotrophic lateral sclerosis (ALS); central motor conduction time; corticomotoneuronal system; D90A CuZn-SOD mutation; magnetic stimulation; multiple sclerosis (MS);
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
64
Recensione:
Indirizzi per estratti:
Indirizzo: Weber, M Vancouver Gen Hosp, Neuromuscular Dis Unit, Willow Pavill,1st Floor,855 W 12th Ave, Vancouver, BC V5Z 1M9, Canada Vancouver Gen Hosp Willow Pavill,1st Floor,855 W 12th Ave Vancouver BC Canada V5Z 1M9
Citazione:
M. Weber et al., "The physiological basis of conduction slowing in ALS patients homozygous for the D90A CuZn-SOD mutation", MUSCLE NERV, 24(1), 2001, pp. 89-97

Abstract

Familial amyotrophic lateral sclerosis (ALS) with the autosomal-recessively inherited D90A CuZn-superoxide dismutase (CuZn-SOD) mutation is characterized by a stereotypic slowly progressive, distinctive phenotype and very slow central motor conduction. To determine the basis of this slowing, we assessed corticomotoneuronal function using peristimulus time histograms (PSTHs) in 8 ALS patients homozygous for the D90A CuZn-SOD mutation. The resultswere compared with findings in 10 patients with multiple sclerosis (MS), in which slowing of central motor conduction is common, and 11 healthy subjects. PSTHs were constructed from 3-7 different, voluntarily recruited motorunits recorded in each patient from the extensor digitorum communis muscle(EDC). In D90A and MS patients, the stimulus threshold, onset latency, number of excess bins, duration, amplitude, and synchrony of the primary peak differed significantly from controls (P < 0.0004). The mean onset latency of the primary peak in D90A patients was 35.3 ms, compared to 23.6 ms for MSpatients and 19.3 ms for normal subjects (P < 0.0001). In the D90A patients, the onset latencies of the primary peak had a bimodal distribution, whereas in MS the distribution showed a continuum. Loss of synchrony was similar in D90A and MS patients, but the threshold, number of excess bins, and duration differed significantly (P < 0.0057), which suggests that either axonal loss or demyelination can result in delayed and desynchronized primary peaks. We propose that conduction slowing in the D90A homozygotes results from selective toss of fast-conducting large pyramidal cells with preservation of slow-conducting mono- or polysynaptic corticomotoneuronal connections. (C) 2001 John Wiley 8 Sons, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 05:15:50