Arquivos de Neuro-Psiquiatria | |
Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases | |
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
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