Pallidal and thalamic deep brain stimulation in myoclonus-dystonia.
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.
Gruber
Doreen
D
Departments of Neurology, University Medicine Berlin, Berlin, Germany.
Kühn
Andrea A
AA
Schoenecker
Thomas
T
Kivi
Anatol
A
Trottenberg
Thomas
T
Hoffmann
Karl-Titus
KT
Gharabaghi
Alireza
A
Kopp
Ute A
UA
Schneider
Gerd-Helge
GH
Klein
Christine
C
Asmus
Friedrich
F
Kupsch
Andreas
A
D000328:Adult; D000368:Aged; D003072:Cognition Disorders / Q000209:etiology; D046690:Deep Brain Stimulation / Q000379:methods*; D020821:Dystonic Disorders / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D005260:Female; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D019964:Mood Disorders / Q000209:etiology; D011788:Quality of Life / Q000523:psychology; D012189:Retrospective Studies; D013788:Thalamus / Q000502:physiology*