title: Deep brain stimulation of globus pallidus internus for DYT1 positive primary generalized dystonia.
abstract: BACKGROUND
  Deep brain stimulation (DBS) of the globus pallidus internus (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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 1. MD., State University of New York, Downstate Medical Center, Department of Neurology, Brooklyn, NY 11203, USA. shz_miri@yahoo.com.
authors.forename: Shahnaz
authors.initials: S
authors.lastname: Miri
authors.affiliation: 2. MD., Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. esighoreyshi@yahoo.com.
authors.forename: Esmaeil
authors.initials: E
authors.lastname: Ghoreyshi
authors.affiliation: 3. MD., Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. gshahidi@hotmail.com.
authors.forename: Gholam Ali
authors.initials: GA
authors.lastname: Shahidi
authors.affiliation: 4. MD., Iran University of Medical Sciences, Department of Neurosurgery, Rasool-Akram Hospital, Tehran, Iran. m_parvaresh@yahoo.com.
authors.forename: Mansour
authors.initials: M
authors.lastname: Parvaresh
authors.affiliation: 5. MD., Assistant Professor of Neurology, Iran University of Medical Sciences, Department of Neurology, Rasool- Akram Hospital, Tehran, Iran. m.roohani@tums.ac.ir ; mohammadroohani@gmail.com.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Rohani
authors.affiliation: 6. MD., Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran. mehdisaffari_hn@yahoo.com.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Saffari
keywords: DYT1 dystonia; Deep brain stimulation; Globus pallidus internus