• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>The Internet Journal of Neurology >Issacs' Syndrome Successfully Treated With Phenytoin At Low Doses
【24h】

Issacs' Syndrome Successfully Treated With Phenytoin At Low Doses

机译：以撒低剂量成功治疗以撒综合征

获取原文
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

We report two patients with Isaacs' syndrome (acquired neuromyotonia) who responded remarkably well clinical and electromyografically to low doses of phenytoin orally. Patients presented with associated common diseases in the former Transkei (South Africa) such as neurocysticercosis, shistosomiasis, tuberculosis and HIV seropositive and we have hypothesized about neuro-immunologic mechanism, and peripheral nerve membrane disorders in these patients and the phenytoin response. Introduction Isaacs' syndrome (IS) is characterized by spontaneously occurring muscle activity of the peripheral nerve origin, which can be triggered by induced muscle contraction or voluntary muscular activity. This uncommon disorder described in 1961 1 in which hyper excitability of peripheral motor nerves resulting in continuous muscle fiber activity leading to incapacitating muscle twitching, cramps, and weakness. Although IS (also known as acquired neuromyotonia) may sometimes accompany hereditary neuropathies or other diseases. IS has been long recognized by several physicians 1 2 3 however its rarity and the variability of its clinical manifestation and ways of presentation is probable the most important reason why its frequently misdiagnosed or wrongly treated 4 Autoimmune etiology had been proposed 5 6 Clinical evidence suggesting a possible autoimmune etiology included the presence of oligoclonal bands in the spinal fluid of some patients and clinical improvement following plasma exchange 6 7 In the first group of 40 patients reported in the past thirty years, thymomas, myasthenia gravis, raised anti-acetylcholine receptor antibody titers among other immunological disorders were indentified 6 and antibodies against potassium channel of peripheral nerves were also found 8 9 10 Phenytoin and cabamazepine have been reported to be efficacious 1 11 Modulation of neuronal sodium channels decreases cellular excitability and diminishes the axonal propagation of nerve impulses, this is a clinically useful mechanism and has been demonstrated to be integral to the activity of major antiepileptic drugs such as carbamazepine and phenytoin 12 Here we report two patients diagnosed as IS and treated with dosage of 100 mg or less of phenytoin orally and observed a remarkable improvement clinical and electromyografically. Case report 1 XhM a 49 year old lady came to special medical clinic complaining, painful inability to open her hands and to extends her toes which were in flexed position 24 hours a day for the past six months after a complete recovery of acute renal failure due to herbal medicine intoxication and defaulted antiepileptic (figure 1). Past medical history a family history of pulmonary tuberculosis, hematuria is mentioned. Apart from cramps and muscle stiffness on the distal region of the limbs she denied any other clinical manifestation currently. On examination mild weakness on the distal regions in the four limbs, bilateral and symmetrical pseudomyotonia in the hands and feet, were observed. EMG before treatment showed doublet, triplet and multiplet single unit discharges with high an irregular intraburst frequency apart from fibrillation potentials. No laboratory tests abnormalities were seen. After a regular oral course of 50 mg of phenyotin twice daily all neuromuscular manifestation disappeared (figure 2) and EMG only showed a few isolated fibrillation potentials
机译：我们报告了两名以撒综合征（后天性神经强直）的患者，对低剂量的苯妥英钠口服后临床和肌电反应明显好。在前特兰斯凯（南非）出现相关常见疾病的患者，例如神经囊虫病，血吸虫病，结核病和HIV血清阳性，我们假设了这些患者的神经免疫机制，周围神经膜疾病和苯妥英钠应答。简介艾萨克斯综合症（IS）的特征在于周围神经起源的自发发生的肌肉活动，其可能由诱导的肌肉收缩或自愿的肌肉活动触发。这种不常见的疾病描述于1961年1，其中周围运动神经的过度兴奋性导致持续的肌纤维活动，从而导致丧失能力的肌肉抽搐，抽筋和无力。虽然IS（也称为获得性神经强直）有时可能会伴随遗传性神经病或其他疾病。长期以来，IS已被几位医生认可1 2 3，但是它的稀有性及其临床表现和表现方式的可变性可能是其经常被误诊或错误治疗的最重要原因4提出了自身免疫病因5 6临床证据表明可能的自身免疫病因包括某些患者的脊髓液中存在寡克隆带以及血浆置换后的临床改善6 7在过去30年中报告的第一批40例患者中，胸腺瘤，重症肌无力引起的抗乙酰胆碱受体抗体滴度升高已鉴定出其他免疫系统疾病6，还发现了针对周围神经钾通道的抗体8 9 10据报道苯妥英和卡马西平是有效的1 11神经元钠通道的调节降低细胞兴奋性并减少神经冲动的轴突传播，这是一种临床上有用的机械m并已被证明是主要抗癫痫药如卡马西平和苯妥英钠的活性不可或缺的药物12在这里，我们报道了两名被诊断为IS且口服苯妥英钠100 mg或更少剂量的患者，并观察到临床和肌移植的显着改善。病例报告1 XhM一位49岁的女士来专科医院就诊，痛苦地无法张开手并伸出脚趾，在完全急性肾衰竭完全康复后的六个月中，她每天24小时处于屈伸姿势草药中毒和默认的抗癫痫药（图1）。过去的病史提到肺结核，血尿的家族史。除了四肢远端区域的抽筋和肌肉僵硬外，她目前否认有任何其他临床表现。检查时，四肢远端区域出现轻度无力，手脚出现双侧对称对称性假肌强直。治疗前的肌电图显示单发，三重和多重发单次放电，除颤动电位外，不规则内发频率高。没有发现实验室检查异常。每天两次定期口服50 mg苯妥英钠的口服疗程后，所有神经肌肉表现都消失了（图2），而EMG仅显示出一些孤立的原纤维形成电位

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号