Miri Shahnaz, Shahidi Gholam Ali, Parvaresh Mansour, Rohani Mohammad
1. Post Doctral Research Fellow, SUNY Downstate Medical Center, Brooklyn, NY 11203 - 2098.
2. Assistant Professor of Neurology Iran University of Medical Sciences, Tehran, Iran.
Med J Islam Repub Iran. 2014 Jun 1;28:39. eCollection 2014.
Little is known about the results of pallidal deep brain stimulation (DBS) in DYT6 dystonia. This will be the first report of DYT6 dystonia treated with pallidal DBS from Iran. A 21 years old male patient with DYT6 dystonia underwent bilateral deep brain stimulation. The target of DBS was the sensorimotor region of the posteroventral globus pallidus internus (GPi). DBS parameters included an amplitude of 2.7 V, frequency of 160 Hz, and pulse width of 90 μs which were adjusted according to the patient's response 12 months after surgery. Treatment outcome was measured by the patient's Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) score. Before surgery, the patient's BFMDRS score was 32. However, BFMDRS score reduced to 7 at one year follow up after surgery (78% improvement of total score). Dystonic symptoms of extremities and mouth completely resolved. Also speech and swallowing function significantly improved. Although previous observations reported a poor to moderate response in speech, we found DBS as an effective treatment not only for dystonic features, but also for speech improvement of DYT6 dystonia.
关于苍白球深部脑刺激（DBS）治疗DYT6型肌张力障碍的结果，目前所知甚少。这将是伊朗首例关于苍白球DBS治疗DYT6型肌张力障碍的报告。一名21岁的男性DYT6型肌张力障碍患者接受了双侧深部脑刺激。DBS的靶点是内侧苍白球腹后感觉运动区（GPi）。DBS参数包括2.7V的振幅、160Hz的频率和90μs的脉冲宽度，术后12个月根据患者反应进行调整。治疗效果通过患者的伯克-法恩-马尔登肌张力障碍评定量表（BFMDRS）评分来衡量。术前，患者的BFMDRS评分为32分。然而，术后一年随访时BFMDRS评分降至7分（总分改善78%）。肢体和口部的肌张力障碍症状完全缓解。言语和吞咽功能也显著改善。尽管之前的观察报告言语反应较差至中等，但我们发现DBS不仅对肌张力障碍特征有效，而且对DYT6型肌张力障碍的言语改善也有效。
