Bilateral Stimulation of the Globus Pallidus Internus to Treat Choreathetosis in Huntington's Disease: Technical Case Report

Hebb, Matthew O.; García, René; Gaudet, Paula; Mendez, Ivar

doi:10.1227/01.neu.0000195068.19801.18

Cited by 86 publications

(56 citation statements)

References 37 publications

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“…78 Case reports evaluating bilateral GPi DBS for HD yielded conflicting results during DBS at 40 Hz; chorea was improved in one subject, 79 but it was poorly controlled in another. 80 Finally, studies examining the effects of the frequency of GPi DBS for subjects with dystonia yielded heterogeneous results. Stimulation at 50 Hz 81 or 80 Hz 82 improved Burke-Fahn-Marsden scale scores better in some subjects than stimulation at frequencies higher than 90 Hz.…”

Section: Dbs Mechanisms In Diseases and Targets Where Lowfrequency Dbmentioning

confidence: 99%

Mechanisms of Deep Brain Stimulation in Movement Disorders as Revealed by Changes in Stimulus Frequency

2008

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Summary: Deep brain stimulation (DBS) is an established treatment for symptoms in movement disorders and is under investigation for symptom management in persons with psychiatric disorders and epilepsy. Nevertheless, there remains disagreement regarding the physiological mechanisms responsible for the actions of DBS, and this lack of understanding impedes both the design of DBS systems for treating novel diseases and the effective tuning of current DBS systems. Currently available data indicate that effective DBS overrides pathological bursts, low frequency oscillations, synchronization, and disrupted firing patterns present in movement disorders, and replaces them with more regularized firing. Although it is likely that the specific mechanism(s) by which DBS exerts its effects varies between diseases and target nuclei, the overriding of pathological activity appears to be ubiquitous. This review provides an overview of changes in motor symptoms with changes in DBS frequency and highlights parallels between the changes in motor symptoms and the changes in cellular activity that appear to underlie the motor symptoms.

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Section: Dbs Mechanisms In Diseases and Targets Where Lowfrequency Dbmentioning

confidence: 99%

Mechanisms of Deep Brain Stimulation in Movement Disorders as Revealed by Changes in Stimulus Frequency

2008

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“…106 The best clinical effects were obtained with high-frequency stimulation (180 Hz), at an amplitude of 2.8 V. Stimulation at a lower frequency (40 Hz) resulted in worsening of the hyperkinetic movements. Body weight, mood, energy, and performance of activities of daily living were also positively affected.…”

Section: Deep Brain Stimulationmentioning

confidence: 95%

Symptomatic Treatment of Huntington Disease

2008

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Summary: Huntington disease (HD) is a progressive heredoneurodegenerative disease manifested by chorea and other hyperkinetic (dystonia, myoclonus, tics) and hypokinetic (parkinsonism) movement disorders. In addition, a variety of psychiatric and behavioral symptoms, along with cognitive decline, contribute significantly to the patient's disability. Because there are no effective neuroprotective therapies that delay the progression of the disease, symptomatic treatment remains the cornerstone of medical management. Several classes of medications have been used to ameliorate the various symptoms of HD, including typical and atypical neuroleptics, dopamine depleters, antidepressants, antiglutamatergic drugs, GABA agonists, antiepileptic medications, acetylcholinesterase inhibitors, and botulinum toxin. Recently, surgical approaches including pallidotomy, deep brain stimulation, and fetal cell transplants have been used for the symptomatic treatment of HD. The selected therapy must be customized to the needs of each patient, minimizing the potential adverse effects. The primary aim of this article is to review the role of the different therapies, both available and investigational, for the treatment of the motor, psychiatric, behavioral, and cognitive symptoms of HD, and to examine their impact on the patient's functionality and quality of life.

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“…In fact, these authors chose low-frequency stimulation with 40 Hz since the effect on bradykinesia was better at lower frequency. Hebb et al [13] reported a reduction of UHDRS scores from about 70 before surgery to about 60 on stimulation after 12 months (and a score of 97 in offstimulation condition at one year). The authors did not use low frequency stimulation due to a poor effect on the chorea.…”

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%

“…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: 99%

See 1 more Smart Citation

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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Bilateral Stimulation of the Globus Pallidus Internus to Treat Choreathetosis in Huntington's Disease: Technical Case Report

Abstract: The clinical benefits of DBS observed in this HD patient were comparable to those reported in other hyperkinetic disorders and demonstrate that pallidal stimulation can provide long-term alleviation of HD-associated choreathetosis.

Cited by 86 publications

References 37 publications

Mechanisms of Deep Brain Stimulation in Movement Disorders as Revealed by Changes in Stimulus Frequency

Mechanisms of Deep Brain Stimulation in Movement Disorders as Revealed by Changes in Stimulus Frequency

Symptomatic Treatment of Huntington Disease

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

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