Department of Neurology, Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville, FL 32611, USA.
J Neurol. 2011 Nov;258(11):2069-74. doi: 10.1007/s00415-011-6075-0. Epub 2011 May 7.
The aim of the study is to determine clinical outcomes in patients undergoing Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) for cranio-facial and cranio-cervical dystonia (Meige) symptoms. A total of 6 patients seen between 2002 and 2010 with cranio-facial and cranio-cervical dystonia symptoms were identified from the University of Florida Institutional Review Board approved database. Patients were videotaped using a standardized protocol, and tapes were randomized and blindly reviewed by a movement disorders neurologist. The Unified Dystonia Rating Scale improved 31.6 ± 23.2% (range: 3.4-63.2%) at 6 months and 63.7 ± 35.3% (range: 6.3-100%) at 12 months. The Burke-Fahn-Marsden Dystonia Rating Scale improved 45.3 ± 29.5% (range: 4.7-75.0%) at 6 months and 61.8 ± 30.9% (range: 16.6-100%) at 12 months. One patient significantly had a very large improvement with little evidence of residual dystonia. Blepharospasm improved in all patients, whereas speech and swallowing did not improve in this cohort. Two patients improved with unilateral GPi-DBS, although one required a contralateral DBS later in the disease course. Two patients were managed with low frequency stimulation (<100 Hz). Two patients had less than 20% benefit. GPi-DBS for cranio-facial and cranio-cervical symptoms is an effective strategy to manage a subset of patients who remain unresponsive to optimized medical management. Unilateral stimulation may be an option for some patients, but it remains unclear whether response to single-sided stimulation will be sustainable. The mixed results of this GPi-DBS case series highlight the need for a careful re-examination of selection criteria, alternative brain targets, and possibly rescue leads for patients who are non-responders to the GPi target.
本研究旨在确定接受苍白球内侧深部脑刺激（GPi-DBS）治疗颅面和颅颈肌张力障碍（Meige）症状的患者的临床结果。从佛罗里达大学机构审查委员会批准的数据库中确定了 6 名 2002 年至 2010 年间接受颅面和颅颈肌张力障碍症状治疗的患者。患者使用标准化方案进行录像，并将录像随机化，由运动障碍神经病学家进行盲法审查。在 6 个月时，统一肌张力障碍评定量表（Unified Dystonia Rating Scale）改善 31.6±23.2%（范围：3.4-63.2%），在 12 个月时改善 63.7±35.3%（范围：6.3-100%）。 Burke-Fahn-Marsden 肌张力障碍评定量表（Burke-Fahn-Marsden Dystonia Rating Scale）在 6 个月时改善 45.3±29.5%（范围：4.7-75.0%），在 12 个月时改善 61.8±30.9%（范围：16.6-100%）。一名患者的改善非常显著，几乎没有残留的肌张力障碍迹象。所有患者的眼睑痉挛均得到改善，而该队列患者的言语和吞咽功能并未改善。两名患者接受单侧 GPi-DBS 治疗后得到改善，尽管一名患者在疾病后期需要对侧 DBS。两名患者采用低频率刺激（<100 Hz）。两名患者获益不足 20%。GPi-DBS 治疗颅面和颅颈症状是一种有效的策略，可以治疗一部分对优化药物治疗无反应的患者。单侧刺激可能是一些患者的选择，但单侧刺激的反应是否可持续仍不清楚。该 GPi-DBS 病例系列的混合结果突出表明，需要仔细重新审查选择标准、替代脑靶标，以及可能为对 GPi 靶点无反应的患者提供挽救性导联。
