Movement Disorders (revue)

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Electrophysiological deterioration over time in patients with huntington's disease

Identifieur interne : 001A10 ( PascalFrancis/Corpus ); précédent : 001A09; suivant : 001A11

Electrophysiological deterioration over time in patients with huntington's disease

Auteurs : Jean-Pascal Lefaucheur ; Isabelle Menard-Lefaucheur ; Patrick Maison ; Sophie Baudic ; Pierre Cesaro ; Marc Peschanski ; Anne-Catherine Bachoud-Levi

Source :

RBID : Pascal:06-0518086

Descripteurs français

English descriptors

Abstract

In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

pA  
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A03   1    @0 Mov. disord.
A05       @2 21
A06       @2 9
A08 01  1  ENG  @1 Electrophysiological deterioration over time in patients with huntington's disease
A11 01  1    @1 LEFAUCHEUR (Jean-Pascal)
A11 02  1    @1 MENARD-LEFAUCHEUR (Isabelle)
A11 03  1    @1 MAISON (Patrick)
A11 04  1    @1 BAUDIC (Sophie)
A11 05  1    @1 CESARO (Pierre)
A11 06  1    @1 PESCHANSKI (Marc)
A11 07  1    @1 BACHOUD-LEVI (Anne-Catherine)
A14 01      @1 Service de Physiologie - Explorations Fonctionnelles, Hôpital Henri Mondor, AP-HP @2 Créteil @3 FRA @Z 1 aut. @Z 2 aut.
A14 02      @1 INSERM U421, IM3, Faculté de Médecine @2 Créteil @3 FRA @Z 1 aut. @Z 3 aut. @Z 4 aut. @Z 5 aut. @Z 6 aut. @Z 7 aut.
A14 03      @1 Service de Pharmacologie Clinique, Hôpital Henri Mondor, AP-HP @2 Créteil @3 FRA @Z 3 aut.
A14 04      @1 Service de Neurologie, Hôpital Henri Mondor, AP-HP @2 Créteil @3 FRA @Z 5 aut. @Z 7 aut.
A20       @1 1350-1354
A21       @1 2006
A23 01      @0 ENG
A43 01      @1 INIST @2 20953 @5 354000158780860080
A44       @0 0000 @1 © 2006 INIST-CNRS. All rights reserved.
A45       @0 32 ref.
A47 01  1    @0 06-0518086
A60       @1 P
A61       @0 A
A64 01  1    @0 Movement disorders
A66 01      @0 USA
C01 01    ENG  @0 In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.
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C03 07  X  SPA  @0 Potencial evocado somatosensorial @5 13
C03 08  X  FRE  @0 Période muette @4 CD @5 96
C03 08  X  ENG  @0 Silent period @4 CD @5 96
C03 09  X  FRE  @0 Stimulation magnétique transcrânienne @4 CD @5 97
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Format Inist (serveur)

NO : PASCAL 06-0518086 INIST
ET : Electrophysiological deterioration over time in patients with huntington's disease
AU : LEFAUCHEUR (Jean-Pascal); MENARD-LEFAUCHEUR (Isabelle); MAISON (Patrick); BAUDIC (Sophie); CESARO (Pierre); PESCHANSKI (Marc); BACHOUD-LEVI (Anne-Catherine)
AF : Service de Physiologie - Explorations Fonctionnelles, Hôpital Henri Mondor, AP-HP/Créteil/France (1 aut., 2 aut.); INSERM U421, IM3, Faculté de Médecine/Créteil/France (1 aut., 3 aut., 4 aut., 5 aut., 6 aut., 7 aut.); Service de Pharmacologie Clinique, Hôpital Henri Mondor, AP-HP/Créteil/France (3 aut.); Service de Neurologie, Hôpital Henri Mondor, AP-HP/Créteil/France (5 aut., 7 aut.)
DT : Publication en série; Niveau analytique
SO : Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 9; Pp. 1350-1354; Bibl. 32 ref.
LA : Anglais
EA : In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.
CC : 002B17; 002B17G; 002B17F
FD : Système nerveux pathologie; Chorée Huntington; Détérioration; Homme; Programme; Réflexe palpébral; Potentiel évoqué somatosensoriel; Période muette; Stimulation magnétique transcrânienne
FG : Encéphale pathologie; Extrapyramidal syndrome; Maladie dégénérative; Maladie héréditaire; Système nerveux central pathologie; Electrophysiologie
ED : Nervous system diseases; Huntington disease; Deterioration; Human; Program; Palpebral reflex; Somatosensory evoked potential; Silent period; Transcranial magnetic stimulation
EG : Cerebral disorder; Extrapyramidal syndrome; Degenerative disease; Genetic disease; Central nervous system disease; Electrophysiology
SD : Sistema nervioso patología; Corea Huntington; Deterioración; Hombre; Programa; Reflejo palpebral; Potencial evocado somatosensorial
LO : INIST-20953.354000158780860080
ID : 06-0518086

Links to Exploration step

Pascal:06-0518086

Le document en format XML

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<div type="abstract" xml:lang="en">In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.</div>
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<ET>Electrophysiological deterioration over time in patients with huntington's disease</ET>
<AU>LEFAUCHEUR (Jean-Pascal); MENARD-LEFAUCHEUR (Isabelle); MAISON (Patrick); BAUDIC (Sophie); CESARO (Pierre); PESCHANSKI (Marc); BACHOUD-LEVI (Anne-Catherine)</AU>
<AF>Service de Physiologie - Explorations Fonctionnelles, Hôpital Henri Mondor, AP-HP/Créteil/France (1 aut., 2 aut.); INSERM U421, IM3, Faculté de Médecine/Créteil/France (1 aut., 3 aut., 4 aut., 5 aut., 6 aut., 7 aut.); Service de Pharmacologie Clinique, Hôpital Henri Mondor, AP-HP/Créteil/France (3 aut.); Service de Neurologie, Hôpital Henri Mondor, AP-HP/Créteil/France (5 aut., 7 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 9; Pp. 1350-1354; Bibl. 32 ref.</SO>
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<EA>In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.</EA>
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