Good outcomes have been reported in deep brain stimulation (DBS) for myoclonus-dystonia syndrome (M-D), a heritable disease characterized by childhood-onset myoclonic jerks and dystonia in the upper body. This meta-analysis was to evaluate the clinical outcomes consecutively, compare the stimulation targets, and identify potential prognostic factors. A systematic literature search was performed on PubMed, Web of Science, and Embase. The primary outcome was the percent improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) scores for dystonia and Unified Myoclonus Rating Scale (UMRS) scores for myoclonus at the last follow-up visit. BFMDRS-disability scores of the patients were also summarized. Pearson correlation analyses were performed to identify the myoclonus and dystonia outcome predictors. Thirty-one studies reporting 71 patients were included. There were significant improvements in BFMDRS-M and BFMDRS-disability scores in each time category and at the last follow-up visit. Mean improvement (%) in UMRS was 79.5 ± 18.2, and 94.1% of the patients showed > 50% improvement in UMRS scores at the last follow-up visit. There was a significant trend toward improved myoclonus outcome with older age at onset and shorter disease duration. Most of the adverse events were mild and transient, and pallidal stimulation seemed to be better with respect to fewer stimulation-induced events. Based on the current data, DBS is effective for even the severe M-D. Surgery at an early stage may predict a better outcome. Although targets do not serve as the outcome predictors, pallidal stimulation may be preferred due to fewer stimulation-induced events.
Keywords: Deep brain stimulation; Globus pallidus internus; Individual patient data; Meta-analysis; Myoclonus dystonia syndrome; Predictive factors; Ventral intermediate nucleus.