Pallidal and thalamic deep brain stimulation in myoclonus-dystonia.
Identifieur interne : 002C44 ( Ncbi/Checkpoint ); précédent : 002C43; suivant : 002C45Pallidal and thalamic deep brain stimulation in myoclonus-dystonia.
Auteurs : Doreen Gruber [Allemagne] ; Andrea A. Kühn ; Thomas Schoenecker ; Anatol Kivi ; Thomas Trottenberg ; Karl-Titus Hoffmann ; Alireza Gharabaghi ; Ute A. Kopp ; Gerd-Helge Schneider ; Christine Klein ; Friedrich Asmus ; Andreas KupschSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2010.
English descriptors
- KwdEn :
- Adult, Aged, Cognition Disorders (etiology), Deep Brain Stimulation (methods), Dystonic Disorders (physiopathology), Dystonic Disorders (psychology), Dystonic Disorders (therapy), Female, Globus Pallidus (physiology), Humans, Magnetic Resonance Imaging (methods), Male, Middle Aged, Mood Disorders (etiology), Quality of Life (psychology), Retrospective Studies, Thalamus (physiology).
- MESH :
- etiology : Cognition Disorders, Mood Disorders.
- methods : Deep Brain Stimulation, Magnetic Resonance Imaging.
- physiology : Globus Pallidus, Thalamus.
- physiopathology : Dystonic Disorders.
- psychology : Dystonic Disorders, Quality of Life.
- therapy : Dystonic Disorders.
- Adult, Aged, Female, Humans, Male, Middle Aged, Retrospective Studies.
Abstract
Deep brain stimulation (DBS) of the internal globus pallidus (GPi) and ventral intermediate thalamic nucleus (VIM) are established treatment options in primary dystonia and tremor syndromes and have been reported anecdotally to be efficacious in myoclonus-dystonia (MD). We investigated short- and long-term effects on motor function, cognition, affective state, and quality of life (QoL) of GPi- and VIM-DBS in MD. Ten MD-patients (nine epsilon-sarcoglycan-mutation-positive) were evaluated pre- and post-surgically following continuous bilateral GPi- and VIM-DBS at four time points: presurgical, 6, 12, and as a last follow-up at a mean of 62.3 months postsurgically, and in OFF-, GPi-, VIM-, and GPi-VIM-DBS conditions by validated motor [unified myoclonus rating scale (UMRS), TSUI Score, Burke-Fahn-Marsden dystonia rating scale (BFMDRS)], cognitive, affective, and QoL-scores. MD-symptoms significantly improved at 6 months post-surgery (UMRS: 61.5%, TSUI Score: 36.5%, BFMDRS: 47.3%). Beneficial effects were sustained at long-term evaluation post-surgery (UMRS: 65.5%, TSUI Score: 35.1%, BFMDRS: 48.2%). QoL was significantly ameliorated; affective status and cognition remained unchanged postsurgically irrespective of the stimulation conditions. No serious long-lasting stimulation-related adverse events (AEs) were observed. Both GPi- and VIM-DBS offer equally effective and safe treatment options for MD. With respect to fewer adverse, stimulation-induced events of GPi-DBS in comparison with VIM-DBS, GPi-DBS seems to be preferable. Combined GPi-VIM-DBS can be useful in cases of incapaciting myoclonus, refractory to GPi-DBS alone.
DOI: 10.1002/mds.23312
PubMed: 20623686
Affiliations:
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pubmed:20623686Le document en format XML
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<front><div type="abstract" xml:lang="en">Deep brain stimulation (DBS) of the internal globus pallidus (GPi) and ventral intermediate thalamic nucleus (VIM) are established treatment options in primary dystonia and tremor syndromes and have been reported anecdotally to be efficacious in myoclonus-dystonia (MD). We investigated short- and long-term effects on motor function, cognition, affective state, and quality of life (QoL) of GPi- and VIM-DBS in MD. Ten MD-patients (nine epsilon-sarcoglycan-mutation-positive) were evaluated pre- and post-surgically following continuous bilateral GPi- and VIM-DBS at four time points: presurgical, 6, 12, and as a last follow-up at a mean of 62.3 months postsurgically, and in OFF-, GPi-, VIM-, and GPi-VIM-DBS conditions by validated motor [unified myoclonus rating scale (UMRS), TSUI Score, Burke-Fahn-Marsden dystonia rating scale (BFMDRS)], cognitive, affective, and QoL-scores. MD-symptoms significantly improved at 6 months post-surgery (UMRS: 61.5%, TSUI Score: 36.5%, BFMDRS: 47.3%). Beneficial effects were sustained at long-term evaluation post-surgery (UMRS: 65.5%, TSUI Score: 35.1%, BFMDRS: 48.2%). QoL was significantly ameliorated; affective status and cognition remained unchanged postsurgically irrespective of the stimulation conditions. No serious long-lasting stimulation-related adverse events (AEs) were observed. Both GPi- and VIM-DBS offer equally effective and safe treatment options for MD. With respect to fewer adverse, stimulation-induced events of GPi-DBS in comparison with VIM-DBS, GPi-DBS seems to be preferable. Combined GPi-VIM-DBS can be useful in cases of incapaciting myoclonus, refractory to GPi-DBS alone.</div>
</front>
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