Chronic stimulation of the globus pallidus internus for treatment of non-DYT1generalized dystonia and choreoathetosis: 2-year follow up
Joachim K. Krauss
Joachim K. Krauss
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
Thomas J. Loher
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
Ralf Weigel
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
H. Holger Capelle
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
Sabine Weber
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
Jean-Marc Burgunder
Departments of Neurology and Neurosurgery, Inselspital, University of Berne, Switzerland; and Department of Neurosurgery, University Hospital, Mannheim, Germany
Object.The authors studied the long-term efficacy of deep brain stimulation (DBS) of the posteroventral lateral globus pallidus internus up to 2 years postoperatively in patients with primary non-DYT1广义肌张力障碍或舞蹈手足徐动症。The results are briefly compared with those reported for DBS inDYT1dystonia (Oppenheim dystonia), which is caused by theDYT1gene.
Methods.Enrollment in this prospective expanded pilot study was limited to adult patients with severely disabling, medically refractory non-DYT1广义肌张力障碍或舞蹈手足徐动症。六consecutive patients underwent follow-up examinations at defined intervals of 3 months, 1 year, and 2 years postsurgery. There were five women and one man, and their mean age at surgery was 45.5 years. Formal assessments included both the Burke-Fahn-Marsden dystonia scale and the recently developed Unified Dystonia Rating Scale. Two patients had primary generalized non-DYT1dystonia, and four suffered from choreoathetosis secondary to infantile cerebral palsy. Bilateral quadripolar DBS electrodes were implanted in all instances, except in one patient with markedly asymmetrical symptoms. There were no adverse events related to surgery.
The Burke-Fahn-Marsden scores in the two patients with generalized dystonia improved by 78 and 71% at 3 months, by 82 and 69% at 1 year, and by 78 and 70% at 2 years postoperatively. This was paralleled by marked amelioration of disability scores. The mean improvement in Burke-Fahn-Marsden scores in patients with choreoathetosis was 12% at 3 months, 29% at 1 year, and 23% at 2 years postoperatively, which was not significant. Two of these patients thought that they had achieved marked improvement at 2 years postoperatively, although results of objective evaluations were less impressive. In these two patients there was a minor but stable improvement in disability scores. All patients had an improvement in pain scores at the 2-year follow-up review. Medication was tapered off in both patients with generalized dystonia and reduced in two of the patients with choreoathetosis. All stimulation-induced side effects were reversible on adjustment of the DBS settings. Energy consumption of the batteries was considerably higher than in patients with Parkinson disease.
Conclusions.Chronic pallidal DBS is a safe and effective procedure in generalized non-DYT1肌张力障碍,它可能成为choic的过程e in patients with medically refractory dystonia. Postoperative improvement of choreoathetosis is more modest and varied, and subjective ratings of outcome may exceed objective evaluations.
Address reprint requests to:Joachim K. Krauss, M.D., Department of Neurosurgery, University Hospital, Klinikum Mannheim, Theodor-Kutzer-Ufer 1–3, 68167 Mannheim, Germany. emailjoachim.krauss@nch.ma.uni-heidelberg.de.
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