Velez-Lago Frances M, Thompson Amanda, Oyama Genko, Hardwick Angela, Sporrer Justin M, Zeilman Pamela, Foote Kelly D, Bowers Dawn, Ward Herbert E, Sanchez-Ramos Juan, Okun Michael S
Departments of Neurology and Neurosurgery, University of Florida, Gainesville, FL 32607, USA.
Stereotact Funct Neurosurg. 2013;91(2):129-33. doi: 10.1159/000341070. Epub 2013 Jan 22.
Huntington's disease (HD) is an autosomal dominant and progressive neurodegenerative syndrome characterized by motor, cognitive and psychiatric manifestations. Chorea and dystonia are features that may be troublesome to some patients and may potentially prove unresponsive to pharmacological treatments. There are several reports on the results of globus pallidus internus deep brain stimulation (DBS) surgery for HD. In these published cases, DBS was utilized mainly to treat disabling chorea. We report our experience with 2 HD cases treated with DBS. The cases illustrate a differential response with a better outcome in the choreic presentation compared to the dystonic presentation. Additionally, DBS worsened gait features in both cases.
亨廷顿舞蹈症（HD）是一种常染色体显性进行性神经退行性综合征，其特征为运动、认知和精神方面的表现。舞蹈症和肌张力障碍是某些患者可能会感到困扰的症状，并且可能对药物治疗无反应。关于苍白球内侧部脑深部电刺激（DBS）手术治疗HD的结果有若干报告。在这些已发表的病例中，DBS主要用于治疗致残性舞蹈症。我们报告了2例接受DBS治疗的HD病例。这些病例显示出不同的反应，舞蹈症表现的治疗效果优于肌张力障碍表现。此外，DBS使两例患者的步态特征均恶化。
