Vesper J, Klostermann F, Funk T, Bock M
Neurochirurgische Klinik und Poliklinik, Universitätsklinikum Benjamin Franklin, Berlin, Germany.
Zentralbl Neurochir. 2002;63(1):18-22. doi: 10.1055/s-2002-31581.
Deep Brain Stimulation (DBS, chronic high frequency stimulation) is well established for Parkinson's disease and tremordominant movement disorders. Generalized dystonia is known as a type of movement disorder in which therapeutic options are very limited. A case of generalized dystonia is reported which was successfully treated by DBS in the Globus pallidus internus (GPI). A 26 years old male suffered from severe torsion dystonia of the lower limbs. The onset of symptoms was at age 7. It started with dystonia of the left foot. He very fast developed severe dystonia of the lower limbs. These complaints were initially treated by diazepam, later by baclofen (Lioresal ((R))) p.o em leader There was no L-DOPA response. Because of the rapid progression of the disease a cervical spinal cord stimulator was implanted with a transient success. Due to further progression of the disease the patient became wheelchair bounded and resistant for oral medication. Limited improvement of symptoms was achieved using continuous intrathecal administration of baclofen. Finally the patient was treated with 980 microgram intrathecal Baclofen (Lioresal ((R))) daily and up to 100 mg diazepam. Under these conditions the patient remained wheelchair bounded with severe lower limb dystonia. As an ultima ratio it was decided to treat the patient with stereotactic implantation of two electrodes (Medtronic 3387) and two neurostimulators (Medtronic ITREL ((R))II). The GPI was the bilateral target point. Intraoperative computerized tomography and ventriculography were used for target setting. Furthermore microrecordings were helpful to ensure the exact electrode positioning. Surgery was performed under sedation. Two weeks after surgery first improvement of symptoms was observed. Patient was able to stand with assistance. At the three months follow-up he could walk without assistance. Slight dystonic movement of the left ankle was the only remaining symptom under stimulation. The oral medication has been continuously reduced. After 6 months it was stopped. The intrathecal administered baclofen was diminished to 250 microgram daily. At the 24 months follow-up the effect of stimulation remained unchanged. However high stimulation parameters are required to maintain an optimal effect (3,5 V, 400 microseconds 145 Hz for both sides). Deep Brain Stimulation of the Globus Pallidus internus is an alternative approach for severe cases of generalized dystonia.
脑深部电刺激术（DBS，慢性高频刺激）在帕金森病和以震颤为主的运动障碍治疗中已得到广泛应用。全身性肌张力障碍是一种治疗选择非常有限的运动障碍类型。本文报道了一例全身性肌张力障碍患者，通过内侧苍白球（GPI）脑深部电刺激术成功治愈。一名26岁男性患有严重的下肢扭转性肌张力障碍。症状始于7岁，最初是左脚肌张力障碍，随后很快发展为严重的下肢肌张力障碍。这些症状最初用安定治疗，后来口服巴氯芬（力奥来素（R）），对左旋多巴无反应。由于疾病进展迅速，植入了颈脊髓刺激器，取得了短暂的成功。由于疾病进一步发展，患者只能依靠轮椅行动，对口服药物产生了耐药性。通过持续鞘内注射巴氯芬，症状得到了有限改善。最后，患者每天鞘内注射980微克巴氯芬（力奥来素（R）），并服用高达100毫克的安定。在这种情况下，患者仍然只能依靠轮椅行动，下肢肌张力障碍严重。作为最后的手段，决定对患者进行立体定向植入两个电极（美敦力3387）和两个神经刺激器（美敦力ITREL（R）II）。双侧靶点为内侧苍白球。术中使用计算机断层扫描和脑室造影进行靶点定位。此外，微电极记录有助于确保电极的精确放置。手术在镇静状态下进行。术后两周观察到症状首次改善，患者在辅助下能够站立。在三个月随访时，他可以独立行走。刺激下仅残留左脚踝轻微的肌张力障碍运动。口服药物持续减少，6个月后停药。鞘内注射的巴氯芬减至每日250微克。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（双侧均为3.5伏、400微秒、145赫兹）。内侧苍白球脑深部电刺激术是治疗严重全身性肌张力障碍病例的一种替代方法。
