Naganuma Ryoji, Yabe Ichiro, Takahashi Ikuko, Matsushima Masaaki, Kano Takahiro, Sasaki Hidenao
Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University.
Department of Neurology, Hokkaido P.W.F.A.C. Obihiro Kosei General Hospital.
Rinsho Shinkeigaku. 2018 Feb 28;58(2):83-87. doi: 10.5692/clinicalneurol.cn-001106. Epub 2018 Jan 31.
In this independent clinical study, we analyzed retrospectively the clinical features of 9 cases (6 male and 3 female) of Lambert-Eaton myasthenic syndrome that were administered 3,4-diaminopyridine (3,4-DAP). Four cases showed no cancer and 5 cases had small cell lung carcinoma. Seven cases were positive for anti voltage-gated calcium channel antibodies. Activities of daily living (ADL) were improved by 3,4-DAP in 8 cases that showed mainly weakness of the extremities, but did not improve ADL in 1 case with cerebellar ataxia of paraneoplastic cerebellar degeneration (PCD). Seven cases showed autonomic symptoms, and 6 cases were improved with 3,4-DAP. The maintenance dose varied widely among individuals, with a single dose ranging from 10 to 40 mg. Each patient was prescribed a maintenance dose 3 to 7 times a day. The daily dosage ranged from 36 to 100 mg. Two cases showed adverse effects to the treatment. Of those 2 cases, 1 case treated at 45 mg/day discontinued treatment, but another case treated at 100 mg/day reduced the dosage and continued treatment. The administration period was 1 to 149 months. Three cases have continued 3,4-DAP for more than 10 years. Four cases have discontinued 3,4-DAP, with 2 cases discontinuing due to death, 1 case discontinuing due to progression of cancer, and 1 case discontinuing due to an adverse reaction. Our results suggest that 3,4-DAP treatment is effective for weakness and autonomic symptoms, but may be ineffective for ataxia of PCD. Treatment with 3,4-DAP can be tolerated for a long period, but the optimal dosage varies widely among individuals.
在这项独立临床研究中，我们回顾性分析了9例接受3,4 - 二氨基吡啶（3,4 - DAP）治疗的兰伯特 - 伊顿肌无力综合征患者（6例男性和3例女性）的临床特征。4例未患癌症，5例患有小细胞肺癌。7例抗电压门控钙通道抗体呈阳性。在主要表现为肢体无力的8例患者中，3,4 - DAP改善了日常生活活动能力（ADL），但在1例伴有副肿瘤性小脑变性（PCD）小脑共济失调的患者中，3,4 - DAP未改善ADL。7例出现自主神经症状，其中6例经3,4 - DAP治疗后得到改善。维持剂量个体差异很大，单次剂量范围为10至40毫克。每位患者每天服用维持剂量3至7次。每日剂量范围为36至100毫克。2例出现治疗不良反应。在这2例中，1例以每日45毫克治疗的患者停止治疗，但另1例以每日100毫克治疗的患者减少剂量并继续治疗。给药期为1至149个月。3例持续服用3,4 - DAP超过10年。4例停止服用3,4 - DAP，其中2例因死亡停药，1例因癌症进展停药，1例因不良反应停药。我们的结果表明，3,4 - DAP治疗对肌无力和自主神经症状有效，但对PCD的共济失调可能无效。3,4 - DAP治疗可长期耐受，但最佳剂量个体差异很大。
