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保留皮质纹状体功能:深部脑刺激亨廷顿氏病。

  Preserving cortico-striatal function deep brain stimulation in Huntington's disease.

Front Syst Neurosci. 2015 Mar 11;9:32. doi: 10.3389/fnsys.2015.00032. eCollection 2015.

  http://www.ncbi.nlm.nih.gov/pubmed/25814939

  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.

  KEYWORDS:

  Huntington’s disease; chorea; cognition; deep brain stimulation; globus pallidus; striatum