Surgical treatment of myoclonus dystonia syndrome.
Identifieur interne : 000762 ( Main/Exploration ); précédent : 000761; suivant : 000763Surgical treatment of myoclonus dystonia syndrome.
Auteurs : Anand I. Rughani [Canada] ; Andres M. LozanoSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2013.
English descriptors
- KwdEn :
- MESH :
- methods : Deep Brain Stimulation.
- physiology : Globus Pallidus, Thalamic Nuclei.
- statistics & numerical data : Databases, Factual.
- therapy : Dystonic Disorders.
- Humans.
Abstract
Myoclonus dystonia (M-D) syndrome is a heritable movement disorder characterized by myoclonic jerks and dystonia primarily of the upper extremities. M-D remains poorly responsive to pharmacological treatment. Emerging reports suggest good response to DBS of the internal globus pallidus (GPi) and ventral intermediate nucleus (VIM) of the thalamus. This study aimed to appraise the value of these two DBS targets by evaluating reports available in the literature. A systematic search of published case reports and case series was performed on Medline and Embase. Responses to DBS were evaluated. Myoclonus was assessed with the Unified Myoclonus Rating Scale (UMRS) and dystonia by the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). The primary outcome of interest was the relative improvements noted with GPi, compared to VIM stimulation. A total of 17 publications yielded 40 unique cases, with mean follow-up of 27.2 months. All patients demonstrated improvements in myoclonus scores, with 93.5% showing at least a 50% improvement in UMRS. The mean improvement in myoclonus scores was 72.6%. In contrast, dystonia scores were improved in 87.9% of patients, with 72.7% reporting at least a 50% improvement in BFMDRS. The mean improvement in dystonia scores was 52.6%. Improvements in myoclonus scores were similar for both GPi (75.7%) and VIM (70.4%; P = 0.27). However, the improvements in dystonia scores were greater with GPi (60.2%), compared to VIM (33.3%; P = 0.03). Although both targets achieve similar improvements in myoclonus, GPi stimulation may be a preferred target because it may achieve greater improvements in dystonia, compared to VIM stimulation.
DOI: 10.1002/mds.25326
PubMed: 23401150
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PubMed, to step Corpus: 000A58
- to stream PubMed, to step Curation: 000A58
- to stream PubMed, to step Checkpoint: 000781
- to stream Ncbi, to step Merge: 003A23
- to stream Ncbi, to step Curation: 003A23
- to stream Ncbi, to step Checkpoint: 003A23
- to stream Main, to step Merge: 000762
- to stream Main, to step Curation: 000762
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Surgical treatment of myoclonus dystonia syndrome.</title><author><name sortKey="Rughani, Anand I" sort="Rughani, Anand I" uniqKey="Rughani A" first="Anand I" last="Rughani">Anand I. Rughani</name><affiliation wicri:level="4"><nlm:affiliation>Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada. anand.rughani@mail.mcgill.ca</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario</wicri:regionArea><orgName type="university">Université de Toronto</orgName><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName></affiliation></author><author><name sortKey="Lozano, Andres M" sort="Lozano, Andres M" uniqKey="Lozano A" first="Andres M" last="Lozano">Andres M. Lozano</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2013">2013</date><idno type="doi">10.1002/mds.25326</idno><idno type="RBID">pubmed:23401150</idno><idno type="pmid">23401150</idno><idno type="wicri:Area/PubMed/Corpus">000A58</idno><idno type="wicri:Area/PubMed/Curation">000A58</idno><idno type="wicri:Area/PubMed/Checkpoint">000781</idno><idno type="wicri:Area/Ncbi/Merge">003A23</idno><idno type="wicri:Area/Ncbi/Curation">003A23</idno><idno type="wicri:Area/Ncbi/Checkpoint">003A23</idno><idno type="wicri:Area/Main/Merge">000762</idno><idno type="wicri:Area/Main/Curation">000762</idno><idno type="wicri:Area/Main/Exploration">000762</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Surgical treatment of myoclonus dystonia syndrome.</title><author><name sortKey="Rughani, Anand I" sort="Rughani, Anand I" uniqKey="Rughani A" first="Anand I" last="Rughani">Anand I. Rughani</name><affiliation wicri:level="4"><nlm:affiliation>Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada. anand.rughani@mail.mcgill.ca</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario</wicri:regionArea><orgName type="university">Université de Toronto</orgName><placeName><settlement type="city">Toronto</settlement><region type="state">Ontario</region></placeName></affiliation></author><author><name sortKey="Lozano, Andres M" sort="Lozano, Andres M" uniqKey="Lozano A" first="Andres M" last="Lozano">Andres M. Lozano</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2013" type="published">2013</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Databases, Factual (statistics & numerical data)</term><term>Deep Brain Stimulation (methods)</term><term>Dystonic Disorders (therapy)</term><term>Globus Pallidus (physiology)</term><term>Humans</term><term>Thalamic Nuclei (physiology)</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Deep Brain Stimulation</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Globus Pallidus</term><term>Thalamic Nuclei</term></keywords><keywords scheme="MESH" qualifier="statistics & numerical data" xml:lang="en"><term>Databases, Factual</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Dystonic Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Humans</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Myoclonus dystonia (M-D) syndrome is a heritable movement disorder characterized by myoclonic jerks and dystonia primarily of the upper extremities. M-D remains poorly responsive to pharmacological treatment. Emerging reports suggest good response to DBS of the internal globus pallidus (GPi) and ventral intermediate nucleus (VIM) of the thalamus. This study aimed to appraise the value of these two DBS targets by evaluating reports available in the literature. A systematic search of published case reports and case series was performed on Medline and Embase. Responses to DBS were evaluated. Myoclonus was assessed with the Unified Myoclonus Rating Scale (UMRS) and dystonia by the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). The primary outcome of interest was the relative improvements noted with GPi, compared to VIM stimulation. A total of 17 publications yielded 40 unique cases, with mean follow-up of 27.2 months. All patients demonstrated improvements in myoclonus scores, with 93.5% showing at least a 50% improvement in UMRS. The mean improvement in myoclonus scores was 72.6%. In contrast, dystonia scores were improved in 87.9% of patients, with 72.7% reporting at least a 50% improvement in BFMDRS. The mean improvement in dystonia scores was 52.6%. Improvements in myoclonus scores were similar for both GPi (75.7%) and VIM (70.4%; P = 0.27). However, the improvements in dystonia scores were greater with GPi (60.2%), compared to VIM (33.3%; P = 0.03). Although both targets achieve similar improvements in myoclonus, GPi stimulation may be a preferred target because it may achieve greater improvements in dystonia, compared to VIM stimulation.</div></front></TEI><affiliations><list><country><li>Canada</li></country><region><li>Ontario</li></region><settlement><li>Toronto</li></settlement><orgName><li>Université de Toronto</li></orgName></list><tree><noCountry><name sortKey="Lozano, Andres M" sort="Lozano, Andres M" uniqKey="Lozano A" first="Andres M" last="Lozano">Andres M. Lozano</name></noCountry><country name="Canada"><region name="Ontario"><name sortKey="Rughani, Anand I" sort="Rughani, Anand I" uniqKey="Rughani A" first="Anand I" last="Rughani">Anand I. Rughani</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000762 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 000762 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Santé
|area= MovDisordV3
|flux= Main
|étape= Exploration
|type= RBID
|clé= pubmed:23401150
|texte= Surgical treatment of myoclonus dystonia syndrome.
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Main/Exploration/RBID.i -Sk "pubmed:23401150" \
| HfdSelect -Kh $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd \
| NlmPubMed2Wicri -a MovDisordV3
| This area was generated with Dilib version V0.6.23. |  |