{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T11:46:51Z","timestamp":1791028011946,"version":"4.1.0"},"reference-count":32,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,8]]},"abstract":"<jats:p content-type=\"fine-print\"><jats:italic>Object.<\/jats:italic> Primary generalized dystonia (PGD) is a medically refractory disease of the brain causing twisting or spasmodic movements and abnormal postures. In more than 30% of cases it is associated with the autosomal <jats:italic>DYT1<\/jats:italic> mutation. Continuous electrical stimulation of the globus pallidus internus (GPi) has been used successfully in the treatment of PGD. The aim of this study was to examine the long-term efficacy and safety of deep brain stimulation (DBS) in the treatment of PGD in children and adults with and without the <jats:italic>DYT1<\/jats:italic> mutation.<\/jats:p>\n<jats:p content-type=\"fine-print\"><jats:italic>Methods.<\/jats:italic> Thirty-one patients with PGD were selected for surgery. Electrodes were bilaterally implanted under stereotactic guidance and connected to neurostimulators that were inserted subcutaneously. Efficacy was evaluated by comparing scores on the clinical and functional Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) before and after implantation.<\/jats:p>\n<jats:p content-type=\"fine-print\">The efficacy of stimulation improved with time. After 2 years, compared with preoperative values, the mean (\u00b1 standard deviation) clinical and functional BFMDRS scores had improved by 79 \u00b1 19% and 65 \u00b1 33%, respectively. At the 2-year follow-up examination the improvement was comparable in patients with and without the <jats:italic>DYT1<\/jats:italic> mutation in both the functional (p = 0.12) and clinical (p = 0.33) scores. Children displayed greater improvements in the clinical score than adult patients (p = 0.04) at 2 years of follow up. In contrast, there was no significant difference in functional scores between children and adults (p = 0.95).<\/jats:p>\n<jats:p content-type=\"fine-print\"><jats:italic>Conclusions.<\/jats:italic> Electrical stimulation of the GPi is an effective, reversible, and adaptable treatment for PGD and should be considered for conditions refractory to pharmaceutical therapies.<\/jats:p>","DOI":"10.3171\/jns.2004.101.2.0189","type":"journal-article","created":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T18:00:51Z","timestamp":1242237651000},"page":"189-194","source":"Crossref","is-referenced-by-count":314,"title":["Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term 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