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• Vol.58 No.2
• CLINICA NEUROL, 58: 83−87, 2018
• Vol.58 No.2 contents

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)