Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results

• DOI: 10.3171/jns.2004.101.2.0189
• Publication Date: 2009-05-13T18:00:51Z
• Authors: Philippe Coubes; Laura Cif; Hassan El Fertit; Simone Hemm; Nathalie Vayssiere; Stephanie Serrat; Marie Christine P...; Sylvie Tuffery; Mireille Claustres; Bernard Echenne; Philippe Frerebeau
• Keywords: Refractory (planetary science); Clinical efficacy

Primary generalized dystonia (PGD) is a medically refractory disease of the brain causing twisting or spasmodic movements and abnormal postures. In more than 30% cases it associated with autosomal DYT1 mutation. Continuous electrical stimulation globus pallidus internus (GPi) has been used successfully in treatment PGD. The aim this study was to examine long-term efficacy safety deep (DBS) PGD children adults without mutation.Thirty-one patients were selected for surgery. Electrodes bilaterally implanted under stereotactic guidance connected neurostimulators that inserted subcutaneously. Efficacy evaluated by comparing scores on clinical functional Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) before after implantation. improved time. After 2 years, compared preoperative values, mean (+/- standard deviation) BFMDRS had 79 +/- 19% 65 33%, respectively. At 2-year follow-up examination improvement comparable mutation both (p = 0.12) 0.33) scores. Children displayed greater improvements score adult 0.04) at years follow up. contrast, there no significant difference between 0.95).Electrical GPi an effective, reversible, adaptable should be considered conditions pharmaceutical therapies.