Movement Disorders (revue)

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Status Dystonicus: Predictors of Outcome and Progression Patterns of Underlying Disease

Identifieur interne : 000185 ( PascalFrancis/Corpus ); précédent : 000184; suivant : 000186

Status Dystonicus: Predictors of Outcome and Progression Patterns of Underlying Disease

Auteurs : Alfonso Fasano ; Lucia Ricciardi ; Anna Rita Bentivoglio ; Carlotta Canavese ; Giovanna Zorzi ; Igor Petrovic ; Nikola Kresojevic ; Vladimir S. Kostic ; Marina Svetel ; Norbert Kovacs ; Istvan Balas ; Agathe Roubertie ; Devendra Mishra ; Paolo Mariotti ; Teresa Temudo ; Nardo Nardocci

Source :

RBID : Pascal:12-0248755

Descripteurs français

English descriptors

Abstract

Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

pA  
A01 01  1    @0 0885-3185
A03   1    @0 Mov. disord.
A05       @2 27
A06       @2 6
A08 01  1  ENG  @1 Status Dystonicus: Predictors of Outcome and Progression Patterns of Underlying Disease
A11 01  1    @1 FASANO (Alfonso)
A11 02  1    @1 RICCIARDI (Lucia)
A11 03  1    @1 BENTIVOGLIO (Anna Rita)
A11 04  1    @1 CANAVESE (Carlotta)
A11 05  1    @1 ZORZI (Giovanna)
A11 06  1    @1 PETROVIC (Igor)
A11 07  1    @1 KRESOJEVIC (Nikola)
A11 08  1    @1 KOSTIC (Vladimir S.)
A11 09  1    @1 SVETEL (Marina)
A11 10  1    @1 KOVACS (Norbert)
A11 11  1    @1 BALAS (Istvan)
A11 12  1    @1 ROUBERTIE (Agathe)
A11 13  1    @1 MISHRA (Devendra)
A11 14  1    @1 MARIOTTI (Paolo)
A11 15  1    @1 TEMUDO (Teresa)
A11 16  1    @1 NARDOCCI (Nardo)
A14 01      @1 Istituto di Neurologia, Università Cattolica del Sacro Cuore @2 Rome @3 ITA @Z 1 aut. @Z 2 aut. @Z 3 aut.
A14 02      @1 AFaR-Fatebenefratelli Association for Biomedical Research, Neuromed Institute @2 Rome @3 ITA @Z 1 aut.
A14 03      @1 Department of Neurosciences, Psychiatry and Anaesthesiological Science, University of Messina @2 Messina @3 ITA @Z 2 aut.
A14 04      @1 Neurological Institute "Carlo Besta @2 Milan @3 ITA @Z 4 aut. @Z 5 aut. @Z 16 aut.
A14 05      @1 Institute of Neurology, Clinical Center of Serbia, School of Medicine @2 Belgrade @3 SRB @Z 6 aut. @Z 7 aut. @Z 8 aut. @Z 9 aut.
A14 06      @1 Department of Neurology, University of Pecs @2 Pecs @3 HUN @Z 10 aut. @Z 11 aut.
A14 07      @1 CHU Montpellier, Hôpital Gui de Chauliac, Service de Neuropédiatrie @2 Montpellier @3 FRA @Z 12 aut.
A14 08      @1 INSERM U827, Institut Universitaire de Recherche Clinique @2 Montpellier @3 FRA @Z 12 aut.
A14 09      @1 Department of Pediatrics, Maulana Azad Medical College and LN Hospital, University of Delhi @2 Delhi @3 IND @Z 13 aut.
A14 10      @1 Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore @2 Rome @3 ITA @Z 14 aut.
A14 11      @1 Unidade de Neuropediatria, Serviço de Pediatria, Hospital Geral de Santo Antonio @2 Porto @3 PRT @Z 15 aut.
A20       @1 783-788
A21       @1 2012
A23 01      @0 ENG
A43 01      @1 INIST @2 20953 @5 354000507771510220
A44       @0 0000 @1 © 2012 INIST-CNRS. All rights reserved.
A45       @0 31 ref.
A47 01  1    @0 12-0248755
A60       @1 P @3 CC
A61       @0 A
A64 01  1    @0 Movement disorders
A66 01      @0 USA
C01 01    ENG  @0 Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.
C02 01  X    @0 002B17
C02 02  X    @0 002B17G
C03 01  X  FRE  @0 Dystonie @5 01
C03 01  X  ENG  @0 Dystonia @5 01
C03 01  X  SPA  @0 Distonía @5 01
C03 02  X  FRE  @0 Pathologie du système nerveux @5 02
C03 02  X  ENG  @0 Nervous system diseases @5 02
C03 02  X  SPA  @0 Sistema nervioso patología @5 02
C03 03  X  FRE  @0 Pronostic @5 09
C03 03  X  ENG  @0 Prognosis @5 09
C03 03  X  SPA  @0 Pronóstico @5 09
C03 04  X  FRE  @0 Midazolam @2 NK @2 FR @5 10
C03 04  X  ENG  @0 Midazolam @2 NK @2 FR @5 10
C03 04  X  SPA  @0 Midazolam @2 NK @2 FR @5 10
C03 05  X  FRE  @0 Pantothenate kinase @2 FE @5 11
C03 05  X  ENG  @0 Pantothenate kinase @2 FE @5 11
C03 05  X  SPA  @0 Pantothenate kinase @2 FE @5 11
C07 01  X  FRE  @0 Transferases @2 FE
C07 01  X  ENG  @0 Transferases @2 FE
C07 01  X  SPA  @0 Transferases @2 FE
C07 02  X  FRE  @0 Enzyme @2 FE
C07 02  X  ENG  @0 Enzyme @2 FE
C07 02  X  SPA  @0 Enzima @2 FE
C07 03  X  FRE  @0 Syndrome extrapyramidal @5 37
C07 03  X  ENG  @0 Extrapyramidal syndrome @5 37
C07 03  X  SPA  @0 Extrapiramidal síndrome @5 37
C07 04  X  FRE  @0 Mouvement involontaire @5 38
C07 04  X  ENG  @0 Involuntary movement @5 38
C07 04  X  SPA  @0 Movimiento involuntario @5 38
C07 05  X  FRE  @0 Pathologie du muscle strié @5 39
C07 05  X  ENG  @0 Striated muscle disease @5 39
C07 05  X  SPA  @0 Músculo estriado patología @5 39
C07 06  X  FRE  @0 Trouble neurologique @5 41
C07 06  X  ENG  @0 Neurological disorder @5 41
C07 06  X  SPA  @0 Trastorno neurológico @5 41
C07 07  X  FRE  @0 Pathologie de l'encéphale @5 42
C07 07  X  ENG  @0 Cerebral disorder @5 42
C07 07  X  SPA  @0 Encéfalo patología @5 42
C07 08  X  FRE  @0 Pathologie du système nerveux central @5 43
C07 08  X  ENG  @0 Central nervous system disease @5 43
C07 08  X  SPA  @0 Sistema nervosio central patología @5 43
N21       @1 191
N44 01      @1 OTO
N82       @1 OTO

Format Inist (serveur)

NO : PASCAL 12-0248755 INIST
ET : Status Dystonicus: Predictors of Outcome and Progression Patterns of Underlying Disease
AU : FASANO (Alfonso); RICCIARDI (Lucia); BENTIVOGLIO (Anna Rita); CANAVESE (Carlotta); ZORZI (Giovanna); PETROVIC (Igor); KRESOJEVIC (Nikola); KOSTIC (Vladimir S.); SVETEL (Marina); KOVACS (Norbert); BALAS (Istvan); ROUBERTIE (Agathe); MISHRA (Devendra); MARIOTTI (Paolo); TEMUDO (Teresa); NARDOCCI (Nardo)
AF : Istituto di Neurologia, Università Cattolica del Sacro Cuore/Rome/Italie (1 aut., 2 aut., 3 aut.); AFaR-Fatebenefratelli Association for Biomedical Research, Neuromed Institute/Rome/Italie (1 aut.); Department of Neurosciences, Psychiatry and Anaesthesiological Science, University of Messina/Messina/Italie (2 aut.); Neurological Institute "Carlo Besta/Milan/Italie (4 aut., 5 aut., 16 aut.); Institute of Neurology, Clinical Center of Serbia, School of Medicine/Belgrade/Serbie (6 aut., 7 aut., 8 aut., 9 aut.); Department of Neurology, University of Pecs/Pecs/Hongrie (10 aut., 11 aut.); CHU Montpellier, Hôpital Gui de Chauliac, Service de Neuropédiatrie/Montpellier/France (12 aut.); INSERM U827, Institut Universitaire de Recherche Clinique/Montpellier/France (12 aut.); Department of Pediatrics, Maulana Azad Medical College and LN Hospital, University of Delhi/Delhi/Inde (13 aut.); Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore/Rome/Italie (14 aut.); Unidade de Neuropediatria, Serviço de Pediatria, Hospital Geral de Santo Antonio/Porto/Portugal (15 aut.)
DT : Publication en série; Courte communication, note brève; Niveau analytique
SO : Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2012; Vol. 27; No. 6; Pp. 783-788; Bibl. 31 ref.
LA : Anglais
EA : Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.
CC : 002B17; 002B17G
FD : Dystonie; Pathologie du système nerveux; Pronostic; Midazolam; Pantothenate kinase
FG : Transferases; Enzyme; Syndrome extrapyramidal; Mouvement involontaire; Pathologie du muscle strié; Trouble neurologique; Pathologie de l'encéphale; Pathologie du système nerveux central
ED : Dystonia; Nervous system diseases; Prognosis; Midazolam; Pantothenate kinase
EG : Transferases; Enzyme; Extrapyramidal syndrome; Involuntary movement; Striated muscle disease; Neurological disorder; Cerebral disorder; Central nervous system disease
SD : Distonía; Sistema nervioso patología; Pronóstico; Midazolam; Pantothenate kinase
LO : INIST-20953.354000507771510220
ID : 12-0248755

Links to Exploration step

Pascal:12-0248755

Le document en format XML

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<name sortKey="Bentivoglio, Anna Rita" sort="Bentivoglio, Anna Rita" uniqKey="Bentivoglio A" first="Anna Rita" last="Bentivoglio">Anna Rita Bentivoglio</name>
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<name sortKey="Canavese, Carlotta" sort="Canavese, Carlotta" uniqKey="Canavese C" first="Carlotta" last="Canavese">Carlotta Canavese</name>
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<inist:fA14 i1="04">
<s1>Neurological Institute "Carlo Besta</s1>
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<s3>ITA</s3>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>16 aut.</sZ>
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<author>
<name sortKey="Zorzi, Giovanna" sort="Zorzi, Giovanna" uniqKey="Zorzi G" first="Giovanna" last="Zorzi">Giovanna Zorzi</name>
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<inist:fA14 i1="04">
<s1>Neurological Institute "Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>16 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Petrovic, Igor" sort="Petrovic, Igor" uniqKey="Petrovic I" first="Igor" last="Petrovic">Igor Petrovic</name>
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<inist:fA14 i1="05">
<s1>Institute of Neurology, Clinical Center of Serbia, School of Medicine</s1>
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<s3>SRB</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
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<name sortKey="Kresojevic, Nikola" sort="Kresojevic, Nikola" uniqKey="Kresojevic N" first="Nikola" last="Kresojevic">Nikola Kresojevic</name>
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<inist:fA14 i1="05">
<s1>Institute of Neurology, Clinical Center of Serbia, School of Medicine</s1>
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<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
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<name sortKey="Kostic, Vladimir S" sort="Kostic, Vladimir S" uniqKey="Kostic V" first="Vladimir S." last="Kostic">Vladimir S. Kostic</name>
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<inist:fA14 i1="05">
<s1>Institute of Neurology, Clinical Center of Serbia, School of Medicine</s1>
<s2>Belgrade</s2>
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<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>9 aut.</sZ>
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<name sortKey="Svetel, Marina" sort="Svetel, Marina" uniqKey="Svetel M" first="Marina" last="Svetel">Marina Svetel</name>
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<inist:fA14 i1="05">
<s1>Institute of Neurology, Clinical Center of Serbia, School of Medicine</s1>
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<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>9 aut.</sZ>
</inist:fA14>
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<name sortKey="Kovacs, Norbert" sort="Kovacs, Norbert" uniqKey="Kovacs N" first="Norbert" last="Kovacs">Norbert Kovacs</name>
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<inist:fA14 i1="06">
<s1>Department of Neurology, University of Pecs</s1>
<s2>Pecs</s2>
<s3>HUN</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Balas, Istvan" sort="Balas, Istvan" uniqKey="Balas I" first="Istvan" last="Balas">Istvan Balas</name>
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<inist:fA14 i1="06">
<s1>Department of Neurology, University of Pecs</s1>
<s2>Pecs</s2>
<s3>HUN</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Roubertie, Agathe" sort="Roubertie, Agathe" uniqKey="Roubertie A" first="Agathe" last="Roubertie">Agathe Roubertie</name>
<affiliation>
<inist:fA14 i1="07">
<s1>CHU Montpellier, Hôpital Gui de Chauliac, Service de Neuropédiatrie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
</inist:fA14>
</affiliation>
<affiliation>
<inist:fA14 i1="08">
<s1>INSERM U827, Institut Universitaire de Recherche Clinique</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Mishra, Devendra" sort="Mishra, Devendra" uniqKey="Mishra D" first="Devendra" last="Mishra">Devendra Mishra</name>
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<inist:fA14 i1="09">
<s1>Department of Pediatrics, Maulana Azad Medical College and LN Hospital, University of Delhi</s1>
<s2>Delhi</s2>
<s3>IND</s3>
<sZ>13 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Mariotti, Paolo" sort="Mariotti, Paolo" uniqKey="Mariotti P" first="Paolo" last="Mariotti">Paolo Mariotti</name>
<affiliation>
<inist:fA14 i1="10">
<s1>Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>14 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Temudo, Teresa" sort="Temudo, Teresa" uniqKey="Temudo T" first="Teresa" last="Temudo">Teresa Temudo</name>
<affiliation>
<inist:fA14 i1="11">
<s1>Unidade de Neuropediatria, Serviço de Pediatria, Hospital Geral de Santo Antonio</s1>
<s2>Porto</s2>
<s3>PRT</s3>
<sZ>15 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
<author>
<name sortKey="Nardocci, Nardo" sort="Nardocci, Nardo" uniqKey="Nardocci N" first="Nardo" last="Nardocci">Nardo Nardocci</name>
<affiliation>
<inist:fA14 i1="04">
<s1>Neurological Institute "Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>16 aut.</sZ>
</inist:fA14>
</affiliation>
</author>
</analytic>
<series>
<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint>
<date when="2012">2012</date>
</imprint>
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<title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
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<term>Dystonia</term>
<term>Midazolam</term>
<term>Nervous system diseases</term>
<term>Pantothenate kinase</term>
<term>Prognosis</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr">
<term>Dystonie</term>
<term>Pathologie du système nerveux</term>
<term>Pronostic</term>
<term>Midazolam</term>
<term>Pantothenate kinase</term>
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<front>
<div type="abstract" xml:lang="en">Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.</div>
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<s1>Department of Neurosciences, Psychiatry and Anaesthesiological Science, University of Messina</s1>
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<s3>ITA</s3>
<sZ>2 aut.</sZ>
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<s1>Neurological Institute "Carlo Besta</s1>
<s2>Milan</s2>
<s3>ITA</s3>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>16 aut.</sZ>
</fA14>
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<s1>Institute of Neurology, Clinical Center of Serbia, School of Medicine</s1>
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<s3>SRB</s3>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
<sZ>9 aut.</sZ>
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<s1>Department of Neurology, University of Pecs</s1>
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<s3>HUN</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
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<s1>CHU Montpellier, Hôpital Gui de Chauliac, Service de Neuropédiatrie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
</fA14>
<fA14 i1="08">
<s1>INSERM U827, Institut Universitaire de Recherche Clinique</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>12 aut.</sZ>
</fA14>
<fA14 i1="09">
<s1>Department of Pediatrics, Maulana Azad Medical College and LN Hospital, University of Delhi</s1>
<s2>Delhi</s2>
<s3>IND</s3>
<sZ>13 aut.</sZ>
</fA14>
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<s1>Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore</s1>
<s2>Rome</s2>
<s3>ITA</s3>
<sZ>14 aut.</sZ>
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<s1>Unidade de Neuropediatria, Serviço de Pediatria, Hospital Geral de Santo Antonio</s1>
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<sZ>15 aut.</sZ>
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<s0>Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.</s0>
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<ET>Status Dystonicus: Predictors of Outcome and Progression Patterns of Underlying Disease</ET>
<AU>FASANO (Alfonso); RICCIARDI (Lucia); BENTIVOGLIO (Anna Rita); CANAVESE (Carlotta); ZORZI (Giovanna); PETROVIC (Igor); KRESOJEVIC (Nikola); KOSTIC (Vladimir S.); SVETEL (Marina); KOVACS (Norbert); BALAS (Istvan); ROUBERTIE (Agathe); MISHRA (Devendra); MARIOTTI (Paolo); TEMUDO (Teresa); NARDOCCI (Nardo)</AU>
<AF>Istituto di Neurologia, Università Cattolica del Sacro Cuore/Rome/Italie (1 aut., 2 aut., 3 aut.); AFaR-Fatebenefratelli Association for Biomedical Research, Neuromed Institute/Rome/Italie (1 aut.); Department of Neurosciences, Psychiatry and Anaesthesiological Science, University of Messina/Messina/Italie (2 aut.); Neurological Institute "Carlo Besta/Milan/Italie (4 aut., 5 aut., 16 aut.); Institute of Neurology, Clinical Center of Serbia, School of Medicine/Belgrade/Serbie (6 aut., 7 aut., 8 aut., 9 aut.); Department of Neurology, University of Pecs/Pecs/Hongrie (10 aut., 11 aut.); CHU Montpellier, Hôpital Gui de Chauliac, Service de Neuropédiatrie/Montpellier/France (12 aut.); INSERM U827, Institut Universitaire de Recherche Clinique/Montpellier/France (12 aut.); Department of Pediatrics, Maulana Azad Medical College and LN Hospital, University of Delhi/Delhi/Inde (13 aut.); Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore/Rome/Italie (14 aut.); Unidade de Neuropediatria, Serviço de Pediatria, Hospital Geral de Santo Antonio/Porto/Portugal (15 aut.)</AF>
<DT>Publication en série; Courte communication, note brève; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2012; Vol. 27; No. 6; Pp. 783-788; Bibl. 31 ref.</SO>
<LA>Anglais</LA>
<EA>Background: Status dystonicus (SD) is a rare, lifethreatening disorder characterized by acute worsening of generalized dystonia. Methods: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. Results: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). Conclusions: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.</EA>
<CC>002B17; 002B17G</CC>
<FD>Dystonie; Pathologie du système nerveux; Pronostic; Midazolam; Pantothenate kinase</FD>
<FG>Transferases; Enzyme; Syndrome extrapyramidal; Mouvement involontaire; Pathologie du muscle strié; Trouble neurologique; Pathologie de l'encéphale; Pathologie du système nerveux central</FG>
<ED>Dystonia; Nervous system diseases; Prognosis; Midazolam; Pantothenate kinase</ED>
<EG>Transferases; Enzyme; Extrapyramidal syndrome; Involuntary movement; Striated muscle disease; Neurological disorder; Cerebral disorder; Central nervous system disease</EG>
<SD>Distonía; Sistema nervioso patología; Pronóstico; Midazolam; Pantothenate kinase</SD>
<LO>INIST-20953.354000507771510220</LO>
<ID>12-0248755</ID>
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