en Neurology Neurology Original Research Original Research <p> <strong> Background </strong>: Deep brain stimulation (DBS) of the globuspallidusinternus (GPi) is recommended as a promising technique for the management of the primary generalized dystonia (PGD) with DYT1 gene mutation. We present the first report of DBS results in Iranian patients with DYT1 positive PGD.<i /> </p><p> <strong> Methods </strong>: Nine patients who suffered from severely disabling DYT1 positive PGD consecutively were recruited for the study between 2008 and 2010. The patients underwent bilateral deep brain stimulation of the GPi in a single procedure. The mean follow up duration was 8.8 ± 2.2 months. The efficacy of the intervention was evaluated by comparing pre- and post operative scores of patients with Burke-Fahn-Marsden's dystonia Scale (BFMDS). Statistical analysis was performed using SPSS 11.0 software. <i /></p><p> <strong> Results: </strong>Of 9 patients six were female with the mean age of 15.2 ± 5.5 years old (range: 8- 25 years old). The mean for Burke-Fahn-Marsden's Dystonia Rating Scale (BFMDRS) score was 47.22 ± 14.1 before surgery and 12.3 ± 8.2 after follow up, which significantly improved (P=0.0001).The mean stimulation parameters at the last visit were at a frequency of 152.2 ± 32.4 Hz (range 130- 230 Hz), a voltage of 2.6 ± 0.7 V (range 1.1- 4), and a pulse width of 60 μsec. No complication was observed during follow up. <i /></p><p> <strong> Conclusion: </strong>Bilateral DBS of the GPi has an encouraging result for the management of DYT1 positive PGD and is recommended as a safe technique for the treatment of these patients. Shorter pulse width in stimulation parameters is suggested for DYT1 dystonia patients. <i /></p> Deep brain stimulation, Globus pallidusinternus, DYT1 dystonia. 119 123 http://mjiri.iums.ac.ir/browse.php?a_code=A-10-1-646&slc_lang=en&sid=1 Shahnaz Miri shz_miri@yahoo.com 200319475328460012965 200319475328460012965 No State University of New York, Downstate Medical Center, Department of Neurology, Brooklyn, NY 11203, USA. Esmaeil Ghoreyshi esighoreyshi@yahoo.com 200319475328460012966 200319475328460012966 No Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. Gholam Ali Shahidi gshahidi@hotmail.com 200319475328460012967 200319475328460012967 No Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. Mansour Parvaresh m_parvaresh@yahoo.com 200319475328460012968 200319475328460012968 No Iran University of Medical Sciences, Department of Neurosurgery, Rasool-Akram Hospital, Tehran, Iran. Mohammad Rohani mohammadroohani@gmail.com 200319475328460012969 200319475328460012969 Yes Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. Mehdi Saffari mehdisaffari_hn@yahoo.com 200319475328460012970 200319475328460012970 No Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran.