Rinsho Shinkeigaku (Clinical Neurology)

Original Article

A retrospective study of the effects of 3,4-diaminopyridine treatment in Lambert-Eaton myasthenic syndrome

Ryoji Naganuma, M.D.1), Ichiro Yabe, M.D., Ph.D.1), Ikuko Takahashi, M.D., Ph.D.1), Masaaki Matsushima, M.D., Ph.D.1), Takahiro Kano, M.D., Ph.D.2) and Hidenao Sasaki, M.D., Ph.D.1)

1)Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University
2)Department of Neurology, Hokkaido P.W.F.A.C. Obihiro Kosei General Hospital

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.
Full Text of this Article in Japanese PDF (349K)

(CLINICA NEUROL, 58: 83|87, 2018)
key words: Lambert-Eaton myasthenic syndrome, 3,4-diaminopyiridine

(Received: 25-Sep-17)