Movement Disorders (revue)

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Stiff man syndrome and related conditions

Identifieur interne : 000686 ( PascalFrancis/Curation ); précédent : 000685; suivant : 000687

Stiff man syndrome and related conditions

Auteurs : Hans-Michael Meinck [Allemagne] ; Philip D. Thompson [Australie]

Source :

RBID : Pascal:02-0583583

Descripteurs français

English descriptors

Abstract

The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/ PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.
pA  
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A03   1    @0 Mov. disord.
A05       @2 17
A06       @2 5
A08 01  1  ENG  @1 Stiff man syndrome and related conditions
A11 01  1    @1 MEINCK (Hans-Michael)
A11 02  1    @1 THOMPSON (Philip D.)
A14 01      @1 Department of Nearology, Im Neuenheimerfeld @2 Heidelberg @3 DEU @Z 1 aut.
A14 02      @1 University Department of Medicine and Department of Neurology, University of Adelaide and Royal Adelaide Hospital @2 Adelaide, South Australia @3 AUS @Z 2 aut.
A20       @1 853-866
A21       @1 2002
A23 01      @0 ENG
A43 01      @1 INIST @2 20953 @5 354000105152380010
A44       @0 0000 @1 © 2002 INIST-CNRS. All rights reserved.
A45       @0 114 ref.
A47 01  1    @0 02-0583583
A60       @1 P
A61       @0 A
A64 01  1    @0 Movement disorders
A66 01      @0 USA
C01 01    ENG  @0 The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/ PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.
C02 01  X    @0 002B17H
C02 02  X    @0 235
C03 01  X  FRE  @0 Homme raide syndrome @5 01
C03 01  X  ENG  @0 Stiff man syndrome @5 01
C03 01  X  SPA  @0 Hombre rígido síndrome @5 01
C03 02  X  FRE  @0 Encéphalomyélite @5 04
C03 02  X  ENG  @0 Encephalomyelitis @5 04
C03 02  X  SPA  @0 Encéfalomielitis @5 04
C03 03  X  FRE  @0 Hypertonie spastique @5 07
C03 03  X  ENG  @0 Spasticity @5 07
C03 03  X  SPA  @0 Hipertonia espástica @5 07
C03 04  X  FRE  @0 Article synthèse @5 16
C03 04  X  ENG  @0 Review @5 16
C03 04  X  SPA  @0 Artículo síntesis @5 16
C03 05  X  FRE  @0 Symptomatologie @5 17
C03 05  X  ENG  @0 Symptomatology @5 17
C03 05  X  SPA  @0 Sintomatología @5 17
C03 06  X  FRE  @0 Diagnostic différentiel @5 18
C03 06  X  ENG  @0 Differential diagnostic @5 18
C03 06  X  SPA  @0 Diagnóstico diferencial @5 18
C03 07  X  FRE  @0 Homme @5 20
C03 07  X  ENG  @0 Human @5 20
C03 07  X  SPA  @0 Hombre @5 20
C07 01  X  FRE  @0 Muscle strié pathologie @5 37
C07 01  X  ENG  @0 Striated muscle disease @5 37
C07 01  X  SPA  @0 Músculo estriado patología @5 37
C07 02  X  FRE  @0 Système nerveux pathologie @5 45
C07 02  X  ENG  @0 Nervous system diseases @5 45
C07 02  X  SPA  @0 Sistema nervioso patología @5 45
C07 03  X  FRE  @0 Encéphale pathologie @5 46
C07 03  X  ENG  @0 Cerebral disorder @5 46
C07 03  X  SPA  @0 Encéfalo patología @5 46
C07 04  X  FRE  @0 Moelle épinière pathologie @5 47
C07 04  X  ENG  @0 Spinal cord disease @5 47
C07 04  X  SPA  @0 Médula espinal patología @5 47
C07 05  X  FRE  @0 Système nerveux central pathologie @5 48
C07 05  X  ENG  @0 Central nervous system disease @5 48
C07 05  X  SPA  @0 Sistema nervosio central patología @5 48
C07 06  X  FRE  @0 Trouble tonus @5 54
C07 06  X  ENG  @0 Muscle tonus alteration @5 54
C07 06  X  SPA  @0 Trastorno tono muscular @5 54
C07 07  X  FRE  @0 Trouble neurologique @5 56
C07 07  X  ENG  @0 Neurological disorder @5 56
C07 07  X  SPA  @0 Trastorno neurológico @5 56
N21       @1 343
N82       @1 PSI

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Pascal:02-0583583

Le document en format XML

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