Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington's disease

Kang, Gail A.; Heath, Susan; Rothlind, Johannes C.; Starr, Philip A.

doi:10.1136/jnnp.2009.202903

Cited by 62 publications

(53 citation statements)

References 28 publications

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“…However, discontinuation of DBS at the last follow-up visit induced reemergence of chorea, supporting a therapeutic effect. Kang et al 26 have also reported sustained chorea reduction 2 years after surgery in 2 patients with HD.…”

Section: Discussionmentioning

confidence: 94%

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Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study

González

Cif

Biolsi

et al. 2014

JNS

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Object To date, experience of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of Huntington's disease (HD) has been limited to a small number of case reports. The aim of this study was to analyze long-term motor outcome of a cohort of HD patients treated with GPi DBS. Methods Seven patients with pharmacologically resistant chorea and functional impairment were included in a prospective open-label study from 2008 to 2011. The main outcome measure was the motor section of the Unified Huntington's Disease Rating Scale. The primary end point was reduction of chorea. Results Patients underwent MRI-guided bilateral GPi implantation. The median duration of follow-up was 3 years. A significant reduction of chorea was observed in all patients, with sustained therapeutic effect; the mean improvement on the chorea subscore was 58.34% at the 12-month follow-up visit (p = 0.018) and 59.8% at the 3-year visit (p = 0.040). Bradykinesia and dystonia showed a nonsignificant trend toward progressive worsening related to disease evolution and partly to DBS. The frequency of stimulation was 130 Hz for all patients. DBS-induced bradykinesia was managed by pulse-width reduction or bipolar settings. Levodopa mildly improved bradykinesia in 4 patients. Regular off-stimulation tests confirmed a persistent therapeutic effect of DBS on chorea. Conclusions GPi DBS may provide sustained chorea improvement in selected HD patients with pharmacologically resistant chorea, with transient benefit in physical aspects of quality of life before progression of behavioral and cognitive disorders. DBS therapy did not improve dystonia or bradykinesia. Further studies including quality of life measures are needed to evaluate the impact of DBS in the long-term outcome of HD.

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Section: Discussionmentioning

confidence: 94%

“…Since the first description of GPi DBS treatment for patients with HD by Moro et al, 28 4 other case reports 4,9,17,26,28,39 have been published, all of them highlighting marked improvement of chorea and variable response in other movement disorders.…”

Section: Discussionmentioning

confidence: 99%

Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study

González

Cif

Biolsi

et al. 2014

JNS

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“…Deep brain stimulation (DBS) has proven its efficacy in other movement disorders such as dystonia and Parkinson's disease, but the interest for applying this technique to HD has been limited [8]. To date, the authors are aware of only 7 cases reported in the literature [9][10][11][12][13][14]. Based on these reports, and the similarity between the symptoms in HD and the choreo-dystonic symptoms seen in PD and dystonia, we decided to perform bilateral pallidal DBS in a patient with HD.…”

Section: Introductionmentioning

confidence: 90%

“…At the evaluation after 4 years the chorea was improved with 56% on stimulation as compared to off stimulation, and motor function improved with 32%. Kang et al [11] reported 2 patients evaluated after 2 years, where chorea improved with 50-60% compared to baseline. Both patients did demonstrate further progression of the disease, including neuropsychological deterioration.…”

Section: Case Presentationmentioning

confidence: 99%

“…The number of HD patients operated during the lesional era, before the introduction of DBS, was limited, and the results seem not to have been encouraging [3][4][5][6][7]. DBS has today largely replaced lesional procedures, and 7 patients with pallidal DBS for HD have recently been presented in 6 different reports [9][10][11][12][13][14]. Moro et al [10] presented one patient in which UHDRS was improved with 31.4 % after 8 months.…”

Section: Case Presentationmentioning

confidence: 99%

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Pallidal Deep Brain Stimulation in the Treatment of Huntington’s Chorea

Loutfi¹,

Lindér²,

Hariz³

et al. 2014

rain Disord Ther

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Despite the success of deep brain stimulation (DBS) in various movement disorders, its use in Huntington´s Disease (HD) has been limited. So far, promising results of pallidal DBS have been reported in 7 patients with HD. We performed bilateral pallidal DBS in a 59 year old woman with HD since 12 years and severe motor symptoms. At the evaluation after 12 months the effect was deemed satisfactory mainly concerning the patient's choreatic symptoms. However, the improvement according to the unified Huntington's disease rating scale was modest, with a score reduction from 92 to 81.

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Huntington Disease and Other Genetic Choreas

Jankovic

2011

Hyperkinetic Movement Disorders

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Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington's disease

Cited by 62 publications

References 28 publications

Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study

Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study

Pallidal Deep Brain Stimulation in the Treatment of Huntington’s Chorea

Huntington Disease and Other Genetic Choreas

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