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J Neurol. 2016 Nov;263(11):2319-2326. Epub 2016 Aug 27.

Long-term results of deep brain stimulation in a cohort of eight children with isolated dystonia.

Author information

1
Department of Neurology, Charité, University Medicine Berlin, Campus Virchow, Berlin, Germany.
2
Department of Neuropediatrics, Charité, University Medicine Berlin, Campus Virchow, Berlin, Germany.
3
Department of Neurology and Stereotactic Neurosurgery, University Medicine of Magdeburg, Magdeburg, Germany.
4
Department of Neurosurgery, Division of Stereotactic Neurosurgery, University Medicine Heidelberg, Heidelberg, Germany.
5
Department of Neurosurgery, Charité, University Medicine Berlin, Campus Virchow, Berlin, Germany.
6
Department of Neurology, Charité, University Medicine Berlin, Campus Virchow, Berlin, Germany. andrea.kuehn@charite.de.

Abstract

Pallidal deep brain stimulation (DBS) is an established treatment for patients with severe isolated dystonia. However, clinical evidence for the long-term use of DBS in children is limited and controlled trials have not yet been conducted. Here, we provide the long-term results of up to 13 years of pallidal DBS in eight pediatric patients with generalized idiopathic or hereditary isolated dystonia (five males, mean age at surgery 12.5 ± 3.5 years), as assessed by retrospective video rating. Video rating was performed at three time points: pre-operative, 1-year short-term follow-up (1y-FU) and long-term last FU (LT-FU, up to 13 years). Symptom severity and disability were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Disability scores were obtained from clinical charts and during the last FU. The mean improvement in BFMDRS motor score was 54.4 ± 8.9 % at 1y-FU and 42.9 ± 11.6 % at LT-FU; the disability scores improved by 59.8 ± 10.3 and 63.3 ± 7.8 %, respectively. Electrode dislocation was noted in one patient and implantable pulse generator dislocation in another, both requiring surgical intervention; no further serious adverse events occurred. Our study presents the first blinded video rating assessment of the short- and long-term effects of pallidal DBS in children with idiopathic or hereditary isolated dystonia. Results confirm that pallidal DBS is a safe and efficacious long-term treatment in children, with overall motor improvement similar to that described in controlled trials in adults.

KEYWORDS:

DYT1 dystonia; Idiopathic dystonia; Long-term effects; Pallidal DBS

PMID:
27567612
DOI:
10.1007/s00415-016-8253-6
[Indexed for MEDLINE]

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