This value is the average age of the patients reported in the article as specific patients could not be identified.This value reflects the gender of the majority of the patients reported in the article as specific patients could not be identified.Please note that this date is based off the date that the article was accepted for publication as the event dates were not provided in the published literature.Section d information references the main component of the system.Other relevant device(s) are: product id: 3389; product id: 3389; product id: neu_ins_stimulator; product id: neu_ins_stimulator.Kosutzka, z., tisch, s., bonnet, c., ruiz, m., hainque, e., welter, ml., viallet, f., karachi, c., navarro, s., jahanshahi, m., rivaud-pechoux, s., grabli, d., roze, e., vidailhet, m.Long-term gpi-dbs improves motor features in myoclonus-dystonia and enhances social adjustment.Mov disord.2018.Doi: 10.1002/mds.27474.If information is provided in the future, a supplemental report will be issued.
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Summary: background: good short-term results of pallidal deep brain stimulation have been reported in myoclonus-dystonia.Efficacy and safety in the long term remain to be established.In addition, the actual impact of dbs treatment on social inclusion is unknown.The objective of this study was to assess the long-term clinical outcome, quality of life, and social adjustment of gpi-dbs in patients with -sarcoglycan (dyt11)-positive myoclonus-dystonia.Methods: consecutive myoclonus-dystonia patients with -sarcoglycan mutations who underwent gpi-dbs were evaluated at least 5 years postoperatively.Motor symptoms were assessed using the burke-fahn-marsden dystonia rating scale including the disability scale, a composite score combining the rest and action parts of the unified myoclonus rating scale and modified abnormal involuntary movement scale.Standardized video-protocols were assessed by a blinded and external movement disorder specialist.Social adjustment, cognition, and mood were evaluated.Results: nine patients (5 women) with long-term gpi- dbs (8.7 ± 3.1 years) were included.There was significant improvement in the composite myoclonus score (94.1% ± 4% improvement; p = 0.008).Dystonia severity was also markedly improved (71.4% ± 28.33% improvement; p = 0.008) as well as motor disability (88.3% ± 20% improvement; p = 0.008) and abnormal involuntary movement score (71.1% ± 15.0% improvement; p = 0.008).No patients experienced postoperative speech or gait problems or any permanent adverse effects.Eight of the 9 patients had fully enhanced social adjustment and personal achievement, with little or no mood or behavioral disorders.Conclusions: gpi-dbs seems to be a safe and efficacious treatment for medically refractory -sarcoglycan myoclonus-dystonia, with sustained motor benefit, good quality of life, and social adjustment in long-term follow-up.Reported events: 1.Patient 5: a (b)(6) male patient with bilateral globus pallidus internus (gpi) deep brain stimulation (dbs) for dystonia experienced an infection at the implantable neurostimulator (ins) site that necessitated device replacement and antibiotic treatment.The patient experienced no permanent neurological sequelae.2.Patient 5: a (b)(6) male patient with bilateral gpi-dbs for dystonia experienced a bicycle accident after which the right electrode was found to be mispositioned and the left electrode was malfunctioning with an open circuit.The leads were replaced.The patient experienced no permanent neurological sequelae.3.Patient 9: a (b)(6) male patient with bilateral gpi-dbs for dystonia experienced an immediate postoperative infection of their left sided ins necessitating device replacement and antibiotic treatment.The patient experienced no permanent neurological sequelae.4.A patient with bilateral gpi-dbs for dystonia and a preoperative history of depression experienced transient depression more than 1 year postoperatively that required hospitalization.All patients were reportedly implanted with 3389 model leads.However, it was not possible to ascertain any additional specific device information from the article or to match the reported event with any previously reported event.
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