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Titolo:
MODELIZATION OF MOTOR-NERVE CONDUCTION VELOCITIES FOR CHARCOT-MARIE-TOOTH (TYPE-1) PATIENTS
Autore:
STURTZ FG; CHAUVIN F; OLLAGNONROMAN E; BOST M; LATOUR P; BONNEBOUCHE C; GONNAUD PM; BADY B; CHAZOT G; VANDENBERGHE A; BEAUVAIS P; CHAPON F; CLAVELOU P; FLOCARD F; MATHIEU M; POUGET J; RENDU M;
Indirizzi:
PROGENITOR INC,1507 CHAMBERS RD COLUMBUS OH 43212 HOP NEUROL,DEPT NEUROL LYON FRANCE CTR ANTICANCEREUX LEON BERARD,DEPT STAT & CLIN EVALUAT LYON FRANCE HOP ANTIQUAILLE,NEUROGENET LAB LYON FRANCE HOP HOTEL DIEU,DEPT GENET MONTREAL PQ CANADA HOP NEUROL,DEPT ELECTROMYOG LYON FRANCE
Titolo Testata:
European neurology
fascicolo: 4, volume: 36, anno: 1996,
pagine: 224 - 228
SICI:
0014-3022(1996)36:4<224:MOMCVF>2.0.ZU;2-H
Fonte:
ISI
Lingua:
ENG
Soggetto:
PERONEAL MUSCULAR-ATROPHY; SENSORY NEUROPATHY TYPE-1; HEREDITARY MOTOR; DISEASE TYPE-1A; DUFFY LOCUS; DUPLICATION; LINKAGE; CHROMOSOME-17; 17P11.2; CMT1A;
Keywords:
HEREDITARY MOTOR AND SENSORY NEUROPATHY TYPE 1; CHROMOSOME 17; NERVE CONDUCTION VELOCITIES; CHARCOT-MARIE-TOOTH SYNDROME; ELECTROPHYSIOLOGICAL MODELIZATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
20
Recensione:
Indirizzi per estratti:
Citazione:
F.G. Sturtz et al., "MODELIZATION OF MOTOR-NERVE CONDUCTION VELOCITIES FOR CHARCOT-MARIE-TOOTH (TYPE-1) PATIENTS", European neurology, 36(4), 1996, pp. 224-228

Abstract

Charcot-Marie-Tooth (CMT) type-1 (CMT1) neuropathy is characterized by peripheral nerve demyelination and has been divided into several subtypes. The most frequent among these, subtype 1A, is related to a microduplication of the region p11.2 of chromosome 17. This region contains the PMP-22 gene which is involved in peripheral nerve myelination. Since motor nerve conduction velocity (MNCV) is closely related to nerve myelination, we compared type-IA patient MNCVs versus non-A CMT1 patient MNCVs, in 57 CMT 1A patients and 21 non-A type-1 patients, Patients with the 17p11.2 duplication have MNCVs that are significantly morereduced (about 20 m/s) compared to patients without the 17p11.2 duplication (about 30 m/s). This study also permits a model of the MNCV in the median nerve (MedMNCV) of CMT1 patients, with age, gender and molecular status as parameters, Furthermore, in order to help clinicians to diagnose subtypes of CMT1 patients, the probability for type 1A is modeled as a function of MedMNCV only.

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Documento generato il 29/11/20 alle ore 02:23:35