Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

Arquivos de Neuro-Psiquiatria

Paulo J. Lorenzoni¹  Rosana H. Scola¹  Cláudia S. Kamoi Kay¹  Sérgio F. Parolin¹  Lineu C. Werneck¹ 

[1] ,Universidade Federal do Paraná Hospital de Clínicas Neuromuscular and Neurology DivisionCuritiba PR ,Brazil

关键词: Lambert-Eaton myasthenic syndrome;    myasthenic syndrome;    P/Q-type voltage-gated calcium channel antibody;    repetitive nerve stimulation;    electrophysiological study;    treatment;    síndrome miastênica de Lambert-Eaton;    síndrome miastênica;    anticorpo anti-canal de cálcio voltagem dependente;    estimulação nervosa repetitiva;    estudo eletrofisiológico;    tratamento;   

DOI  :  10.1590/S0004-282X2010000600004

来源: SciELO

【 摘 要 】

Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the presynaptic neuromuscular transmission, which more frequently occurs as the remote effect of a neoplasm, in the paraneoplastic form (P-LEMS), or in a non-paraneoplastic form (NP-LEMS); but few studies describe the clinical features of NP-LEMS. We analyzed the clinical manifestations, laboratory findings, electrophysiological studies, and treatment responses in ten Brazilian patients suffering from NP-LEMS. The mean age was 41.5 years. More often neurological findings were hyporeflexia or areflexia with a post-exercise improvement. Treatment response occurred with pyridostigmine, guanidine, prednisone, azathioprine, and cyclosporine; but not response was observed after intravenous immunoglobulin and plasma exchange. Age at onset, clinical manifestations, and electrophysiological abnormalities can help more in the diagnosis than serum antibodies; the symptomatic treatment with pyridostigmine was effective; and the immunosuppressive treatment with prednisone, azathioprine, or cyclosporine was more beneficial than plasma exchange or intravenous immunoglobulin treatment.

【 授权许可】

CC BY   
 All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License