Miri Shahnaz, Ghoreyshi Esmaeil, Shahidi Gholam Ali, Parvaresh Mansour, Rohani Mohammad, Saffari Mehdi
1. MD., State University of New York, Downstate Medical Center, Department of Neurology, Brooklyn, NY 11203, USA.
2. MD., Iran University of Medical Sciences, Department of Neurology, Rasool-Akram Hospital, Tehran, Iran.
Med J Islam Repub Iran. 2014 Mar 1;28:18. eCollection 2014.
Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is recommended as a promising technique for the management of the primary generalized dystonia (PGD) with DYT1 gene mutation. We present the first report of DBS results in Iranian patients with DYT1 positive PGD.
Nine patients who suffered from severely disabling DYT1 positive PGD consecutively were recruited for the study between 2008 and 2010. The patients underwent bilateral deep brain stimulation of the GPi in a single procedure. The mean follow up duration was 8.8 ± 2.2 months. The efficacy of the intervention was evaluated by comparing pre- and post operative scores of patients with Burke-Fahn-Marsden's dystonia Scale (BFMDS). Statistical analysis was performed using SPSS 11.0 software.
Of 9 patients six were female with the mean age of 15.2 ± 5.5 years old (range: 8- 25 years old). The mean for Burke-Fahn-Marsden's Dystonia Rating Scale (BFMDRS) score was 47.22 ± 14.1 before surgery and 12.3 ± 8.2 after follow up, which significantly improved (P=0.0001).The mean stimulation parameters at the last visit were at a frequency of 152.2 ± 32.4 Hz (range 130- 230 Hz), a voltage of 2.6 ± 0.7 V (range 1.1- 4), and a pulse width of 60 μsec. No complication was observed during follow up.
Bilateral DBS of the GPi has an encouraging result for the management of DYT1 positive PGD and is recommended as a safe technique for the treatment of these patients. Shorter pulse width in stimulation parameters is suggested for DYT1 dystonia patients.
内侧苍白球（GPi）的脑深部电刺激（DBS）被推荐为一种有前景的技术，用于治疗携带DYT1基因突变的原发性全身性肌张力障碍（PGD）。我们首次报告了伊朗DYT1阳性PGD患者的DBS治疗结果。
2008年至2010年间，连续招募了9名患有严重致残性DYT1阳性PGD的患者进行研究。患者在一次手术中接受了双侧GPi脑深部电刺激。平均随访时间为8.8±2.2个月。通过比较患者术前和术后的伯克-法恩-马斯登肌张力障碍量表（BFMDS）评分来评估干预效果。使用SPSS 11.0软件进行统计分析。
9名患者中6名是女性，平均年龄为15.2±5.5岁（范围：8 - 25岁）。伯克-法恩-马斯登肌张力障碍评定量表（BFMDRS）评分术前平均为47.22±14.1，随访后为12.3±8.2，有显著改善（P = 0.0001）。最后一次随访时的平均刺激参数为频率152.2±32.4赫兹（范围130 - 230赫兹），电压2.6±0.7伏（范围1.1 - 4），脉冲宽度60微秒。随访期间未观察到并发症。
双侧GPi DBS治疗DYT1阳性PGD取得了令人鼓舞的结果，被推荐为治疗这些患者的安全技术。建议DYT1肌张力障碍患者在刺激参数中采用较短的脉冲宽度。
