Vesper J, Klostermann F, Funk Th, Stockhammer F, Brock M
Department of Neurosurgery, University Medical Center Benjamin Franklin, Berlin, Germany.
Acta Neurochir Suppl. 2002;79:83-8. doi: 10.1007/978-3-7091-6105-0_19.
Generalized dystonia is known as a type of movement disorder in which pharmacotherapeutic options are very limited. Deep Brain Stimulation (DBS) is well established for Parkinson's disease (PD) and tremor dominant movement disorders. We report on two cases of generalized dystonia which were successfully treated by chronic high frequency stimulation in the Globus pallidus internus (GPI). Two 26 and 27 years old males suffered from severe torsion dystonia and multisegmental dystonia of the lower limbs. Case 1 is a familiar type of dystonia (DYT1 positive). The onset of symptoms in both cases was at age 7. The complaints were initially treated with orally administered benzodiazepines, anticholinergic drugs, later by baclofen and L-DOPA. However there was no response. Case 2 was a patient with a history of left side dominated dystonia since the age of 8. It was first diagnosed as a psychogenic movement disorder. Prior to surgery he was treated with L-DOPA, anticholinergics, Baclofen without any effect. There was only a limited effect on high doses of diazepam. The patient is DYT1 negative. The target point was on both sides the GPI. Intraoperative computerized tomography (CT) and ventriculography (VG) were used for target setting. Furthermore microrecordings were helpful to ensure the exact electrode position. Surgery was performed under analgosedation. Two weeks after surgery we first observed a relief of symptoms in both cases. A significant reduction in the Burke-Fahn-Marsden-Dystonia Movement Rating Scale was observed at the 6 month follow-up (case 1: 95%, case 2: 80%). In case 1 a slight dystonic movement of the left ankle was the only remaining symptom under stimulation. The medication was continuously reduced. At the 24 month follow-up the effect of stimulation remained unchanged. However high stimulation parameters are required to maintain an optimal effect (mean 3.5 V, 400 microseconds, 145 Hz).
全身性肌张力障碍是一种运动障碍，其药物治疗选择非常有限。深部脑刺激（DBS）已被广泛应用于帕金森病（PD）和震颤为主的运动障碍。我们报告了两例全身性肌张力障碍患者，通过内侧苍白球（GPI）的慢性高频刺激成功治疗。两名分别为26岁和27岁的男性患有严重的扭转性肌张力障碍和下肢多节段肌张力障碍。病例1为家族性肌张力障碍类型（DYT1阳性）。两例患者症状均始于7岁。最初症状采用口服苯二氮䓬类药物、抗胆碱能药物治疗，后来使用巴氯芬和左旋多巴治疗。然而均无反应。病例2是一名自8岁起就有左侧为主的肌张力障碍病史的患者。最初被诊断为精神性运动障碍。手术前，他接受了左旋多巴、抗胆碱能药物、巴氯芬治疗，但均无效果。高剂量地西泮仅有有限的效果。该患者DYT1阴性。靶点均为双侧GPI。术中使用计算机断层扫描（CT）和脑室造影（VG）进行靶点定位。此外，微电极记录有助于确保电极的精确位置。手术在镇痛镇静下进行。术后两周我们首次观察到两例患者症状均有所缓解。在6个月随访时，Burke-Fahn-Marsden肌张力障碍运动评分量表有显著降低（病例1：95%，病例2：80%）。病例1在刺激下仅残留左脚踝轻微的肌张力障碍运动。药物持续减量。在24个月随访时，刺激效果保持不变。然而需要高刺激参数来维持最佳效果（平均3.5伏，400微秒，145赫兹）。
