Abstract
ObjectiveObservational study to evaluate the long-term motor and non-motor effects of deep brain stimulation (DBS) of the globus pallidus internus (GPi) on medically refractory dystonia.BackgroundDystonia is a chronic disease affecting mainly young patients with a regular life expectancy and lifelong need for therapy. Pallidal DBS is an established treatment for severe isolated dystonia but long-term data are sparse.MethodsWe considered 36 consecutive patients with isolated generalized (n = 14) and cervical/segmental (n = 22) dystonia operated at Charité-University Hospital between 2000 and 2007 in a retrospective analysis for long-term outcome of pallidal DBS. In 19 of these patients, we could analyze dystonic symptoms and disability rated by the Burke–Fahn–Marsden Dystonia Rating scale (BFMDRS) at baseline, short-term (ST-FU, range 3–36 months) and long-term follow-up (LT-FU, range 93–197 months). Quality of life and mood were evaluated using the SF36 and Beck Depression Index (BDI) questionnaires.ResultsPatients reached an improvement in motor symptoms of 63.8 ± 5.7% (mean ± SE) at ST-FU and 67.9 ± 6.1% at LT-FU. Moreover, a significant and stable reduction in disability was shown following DBS (54.2 ± 9.4% at ST-FU and 53.8 ± 9.2% at LT-FU). BDI and SF36 had improved by 40% and 23%, respectively, at LT-FU (n = 14). Stimulation-induced adverse events included swallowing difficulties, dysarthria, and bradykinesia. Pulse generator (n = 3) and electrodes (n = 5) were revised in seven patients due to infection.ConclusionsPallidal DBS is a safe and efficacious long-term treatment for dystonia with sustained effects on motor impairment and disability, accompanied by a robust improvement in mood and quality of life.
Highlights
Isolated dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contractions causing twisting movements and abnormal postures that may be tremulous [1, 2]
The aim of the present study was to evaluate long-term effects of bilateral pallidal deep brain stimulation (DBS) on motor symptoms, mood, and quality of life in a cohort of 36 consecutive patients with isolated generalized, segmental, or cervical dystonia operated at Charité Hospital, University Medicine Berlin between 2000 and 2007
Thirty-six patients (22 male; mean age at surgery 49 ± 13 years [mean ± standard deviation], range 20–74 years; mean age of onset 36.3 ± 17 years, range 3–60 years; mean disease duration 12.9 ± 9.9 years, range 2–43 years) meeting the criteria for isolated idiopathic or hereditary dystonia [1, 2] were invited for long-term follow-up (LT-FU)
Summary
Isolated dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contractions causing twisting movements and abnormal postures that may be tremulous [1, 2]. Journal of Neurology (2020) 267:1622–1631 etiologies of dystonia for up to 10 years [8, 9] or shorter follow-up periods [10, 11]. The impact of DBS on quality of life (QoL) or mood has rarely been studied [12, 13]. Detailed analysis of SF36 in larger cohorts 3 and 5 years after stimulation showed heterogeneous effects of DBS on QoL with stable positive effects on health-related physical domains of the questionnaire, but considerably minor effects on mental subdomains. The aim of the present study was to evaluate long-term effects of bilateral pallidal DBS on motor symptoms, mood, and quality of life in a cohort of 36 consecutive patients with isolated generalized, segmental, or cervical dystonia operated at Charité Hospital, University Medicine Berlin between 2000 and 2007
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