Long-term outcome of deep brain stimulation in generalised dystonia: a series of 60 cases

Abstract

Background There is solid evidence of the long term efficacy of deep brain stimulation of the globus pallidus pars interna in the treatment of generalised dystonia. However there are conflicting reports concerning whether certain subgroups gain more benefit from treatment than others. We analysed the results of a series of 60 cases to evaluate the effects of previously proposed prognostic factors including dystonia aetiology, dystonia phenotype, age at onset of dystonia, and duration of dystonia prior to treatment. Methods 60 patients with medically intractable primary or secondary generalised dystonia were treated with deep brain stimulation of the globus pallidus pars interna during the period 1999-2010 at the Department of Neurosurgery in Oxford, UK. Patients were assessed using the Burke-Fahn-Marsden (BFM) Dystonia Rating Scale prior to surgery, 6 months after implantation and thereafter at 1 year, 2 years and 5 years follow-up. Results The group showed mean improvements in the BFM severity and disability scores of 43% and 27%, respectively, by 6 months, and this was sustained. The results in 11 patients with DYT gene mutations were significantly better than in non-genetic primary cases. The results in 12 patients with secondary dystonia were not as good as those seen in non-genetic primary cases but there remained a significant beneficial effect. Age of onset of dystonia, duration of disease prior to surgery, and myoclonic versus torsional disease phenotype had no significant effect on outcome. Conclusions The aetiology of dystonia was the sole factor predicting a better or poorer outcome from globus pallidus pars interna stimulation in this series of patients with generalised dystonia. However even the secondary cases that responded the least well had a substantial reduction in BFM scores compared with preoperative clinical assessments, and these patients should still be considered for deep brain stimulation.

Distonili Bir Hastada Derin Beyin Stimülasyon Cerrahisinin Mobilite, Emosyonel Durum ve Yaşam Kalitesi Üzerine Olan Etkisinin İncelenmesi: Olgu Sunumu

“…The group showed mean improvements in the BFM severity and disability scores of 43% and 27%, respectively, by 6 months, and this was sustained 14 .…”

Langzeiteffekte der tiefen Hirnstimulation bei Bewegungsstörungen

“…Die Besserung im Behinderungs-Teil der Burke-Fahn-Marsden-Skala (BFMDRS-D) fiel in den beiden größ-ten Serien (n=40, davon n=26 generalisiert; n=60 generalisiert) mit 33 bzw. 47% geringer aus [9,30]. Mehrheitlich wurde ein besseres Ansprechen von Patienten mit DYT1-Mutation beschrieben [9].…”

“…47% geringer aus [9,30]. Mehrheitlich wurde ein besseres Ansprechen von Patienten mit DYT1-Mutation beschrieben [9]. Vorbestehende Sprech-und Schluckstö-rungen wurden durch die Stimulation kaum gebessert [25].…”

“…In einer Serie aus New York mit 47 Patienten wurden immerhin 23% Reoperationen erforderlich [18]. Infektionen traten vorwiegend beim Schrittmacherwechsel auf [9]. In einigen Studien wurde über das Auftreten von ParkinsonSymptomen (A-/Bradykinesie, Gangstö-rung z. T. mit Freezing) berichtet.…”

“…Diese stellten auch die Hauptnebenwirkungen in anderen Studien dar. Technische Defekte (18%) und Infektionen (13%) addierten sich in der größten Serie mit 60 Patienten ähnlich hoch [9]. Infektionen ließen sich z. T. rein antibiotisch behandeln und erforderten in anderen Fällen die Explantation des infi-100% 4 5 6 7 8 9 10 (Jahre) 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% Panov et al Abb.…”