<b><i>Introduction:</i></b> Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an efficient treatment of primary dystonia. Few studies have reported the effect of STN-DBS on secondary or acquired dystonia. <b><i>Methods:</i></b> We reported 2 patients with acquired dystonia treated by subthalamic DBS and followed up for 24 months, besides providing a systematic review and meta-analysis of published series. <b><i>Results/Conclusions:</i></b> Both patients had thalamic vascular or autoimmune lesions within the ventral and the pulvinar nuclei. A reduction of 67.2% on the Burke-Fahn-Marsden Dystonia Rating Scale and 90% improvement in disability scores were shown in the first patient, while the second patient showed a lower reduction in both dystonia symptoms (28.6%) and disability scores (44%). Both patients had a significant mean improvement in the quality of life (62.5% in the first and 57.9% in the second) and were free of drugs postoperatively. A systematic review showed a mean follow-up of 13 months in 19 patients, including our 2 patients. The review showed a significant Burke-Fahn-Marsden Dystonia Scale (BFMDRS) score median reduction of 19 points (52.4%; confidence interval [CI]: 11.0–25.0) and a significant median reduction of 6 points in disability scores (44.5%; 95% CI: 4.0–14.0), thereby improving quality of life. Age at surgery was inversely correlated with postoperative improvement (<i>r</i> = 0.63; <i>p</i> = 0.039). Hemidystonia had a nonsignificant better improvement than generalized dystonia (55.3 vs. 43.5%; <i>p</i> = 0.4433). No association between etiology and postoperative improvement and no serious complications were found. Although few data reported so far, subthalamic DBS is likely efficient for acquired dystonia.
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