van Hilten B J, van de Beek W J, Hoff J I, Voormolen J H, Delhaas E M
Department of Neurology, Leiden University Medical Center, The Netherlands.
N Engl J Med. 2000 Aug 31;343(9):625-30. doi: 10.1056/NEJM200008313430905.
Patients with reflex sympathetic dystrophy (also known as the complex regional pain syndrome) may have dystonia, which is often unresponsive to treatment. Some forms of dystonia respond to the intrathecal administration of baclofen, a specific gamma-aminobutyric acid-receptor (type B) agonist that inhibits sensory input to the neurons of the spinal cord. We evaluated this treatment in seven women who had reflex sympathetic dystrophy with multifocal or generalized tonic dystonia. First, we performed a double-blind, randomized, controlled crossover trial of bolus intrathecal injections of 25, 50, and 75 microg of baclofen and placebo. Changes in the severity of dystonia were assessed by the woman and by an investigator after each injection. In the second phase of the study, six of the women received a subcutaneous pump for continuous intrathecal administration of baclofen and were followed for 0.5 to 3 years.
In six women, bolus injections of 50 and 75 microg of baclofen resulted in complete or partial resolution of focal dystonia of the hands but little improvement in dystonia of the legs. During continuous therapy, three women regained normal hand function, and two of these three women regained the ability to walk (one only indoors). In one woman who received continuous therapy, the pain and violent jerks disappeared and the dystonic posturing of the arm decreased. In two women the spasms or restlessness of the legs decreased, without any change in the dystonia.
In some patients, the dystonia associated with reflex sympathetic dystrophy responds markedly to intrathecal baclofen.
反射性交感神经营养不良（也称为复杂性区域疼痛综合征）患者可能出现肌张力障碍，且这种情况通常对治疗无反应。某些形式的肌张力障碍对鞘内注射巴氯芬有反应，巴氯芬是一种特异性γ-氨基丁酸受体（B型）激动剂，可抑制脊髓神经元的感觉输入。我们对7名患有反射性交感神经营养不良并伴有多灶性或全身性强直性肌张力障碍的女性进行了这种治疗评估。首先，我们进行了一项双盲、随机、对照交叉试验，鞘内推注25、50和75微克的巴氯芬及安慰剂。每次注射后，由女性患者和一名研究者评估肌张力障碍严重程度的变化。在研究的第二阶段，6名女性接受了皮下泵持续鞘内注射巴氯芬，并随访0.5至3年。
在6名女性中，推注50和75微克巴氯芬后，手部局灶性肌张力障碍完全或部分缓解，但腿部肌张力障碍改善甚微。在持续治疗期间，3名女性恢复了正常的手部功能，其中2名女性恢复了行走能力（1名仅能在室内行走）。在1名接受持续治疗的女性中，疼痛和剧烈抽搐消失，手臂的肌张力障碍姿势减轻。在2名女性中，腿部的痉挛或不安减轻，肌张力障碍无任何变化。
在一些患者中，与反射性交感神经营养不良相关的肌张力障碍对鞘内注射巴氯芬有明显反应。
