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Published on: March 8, 2015
Preserving cortico-striatal function: deep brain stimulation in Huntington's disease
Sean J Nagel1, Andre G Machado1, John T Gale2
1Cleveland Clinic, Neurologic Institute, Center for Neurological Restoration Cleveland, Ohio, USA ; Department of Neurosurgery, Cleveland Clinic, Neurologic Institute Cleveland, Ohio, USA.
Insights
Deep brain stimulation (DBS) shows potential for treating Huntington's disease (HD) symptoms beyond chorea, including cognitive decline. Further clinical trials are recommended to explore DBS as a disease-modifying therapy for HD patients.
Area of Science:
- Neuroscience
- Neurology
- Genetics
Background:
- Huntington's disease (HD) is an incurable, progressive neurodegenerative disorder.
- Current treatments for HD primarily manage symptoms, lacking disease-modifying capabilities.
- The underlying pathogenesis of HD is understood, yet a cure remains elusive.
Purpose of the Study:
- To explore the potential of deep brain stimulation (DBS) as a therapeutic intervention for Huntington's disease.
- To evaluate DBS's efficacy in addressing not only motor symptoms like chorea but also cognitive and psychiatric disturbances in HD.
- To advocate for new clinical trials investigating DBS for HD.
Main Methods:
- Review of existing literature on DBS in neurodegenerative disorders, including case reports in HD.
- Analysis of the proposed mechanisms of action for DBS in neurological conditions.
- Consideration of DBS's established safety and reversibility profile.
Main Results:
- DBS has demonstrated variable success as a palliative treatment for debilitating chorea in select HD cases.
- Emerging evidence suggests DBS may influence cognitive deterioration, a key feature of HD.
- DBS is a widely used, reversible, and relatively low-risk intervention.
Conclusions:
- Deep brain stimulation (DBS) presents a promising avenue for novel Huntington's disease (HD) therapies.
- Further clinical investigations are warranted to rigorously assess the therapeutic potential of DBS for HD.
- DBS could offer a disease-modifying approach to improve function and alleviate suffering in HD patients.
Abstract:
Huntington's disease (HD) is an incurable neurodegenerative disease characterized by the triad of chorea, cognitive dysfunction and psychiatric disturbances. Since the discovery of the HD gene, the pathogenesis has been outlined, but to date a cure has not been found. Disease modifying therapies are needed desperately to improve function, alleviate suffering, and provide hope for symptomatic patients. Deep brain stimulation (DBS), a proven therapy for managing the symptoms of some neurodegenerative movement disorders, including Parkinson's disease, has been reported as a palliative treatment in select cases of HD with debilitating chorea with variable success. New insights into the mechanism of action of DBS suggest it may have the potential to circumvent other manifestations of HD including cognitive deterioration. Furthermore, because DBS is already widely used, reversible, and has a risk profile that is relatively low, new studies can be initiated. In this article we contend that new clinical trials be considered to test the effects of DBS for HD.
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