Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases
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 clinica...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Academia Brasileira de Neurologia (ABNEURO)
2010-12-01
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| Series: | Arquivos de Neuro-Psiquiatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000600004&lng=en&tlng=en |
| _version_ | 1818499860193607680 |
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| author | Paulo J. Lorenzoni Rosana H. Scola Cláudia S. Kamoi Kay Sérgio F. Parolin Lineu C. Werneck |
| author_facet | Paulo J. Lorenzoni Rosana H. Scola Cláudia S. Kamoi Kay Sérgio F. Parolin Lineu C. Werneck |
| author_sort | Paulo J. Lorenzoni |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-10T20:35:18Z |
| format | Article |
| id | doaj.art-52e49de1d8f64bcfaaf33aaee35b82bf |
| institution | Directory Open Access Journal |
| issn | 1678-4227 |
| language | English |
| last_indexed | 2024-12-10T20:35:18Z |
| publishDate | 2010-12-01 |
| publisher | Academia Brasileira de Neurologia (ABNEURO) |
| record_format | Article |
| series | Arquivos de Neuro-Psiquiatria |
| spelling | doaj.art-52e49de1d8f64bcfaaf33aaee35b82bf2022-12-22T01:34:33ZengAcademia Brasileira de Neurologia (ABNEURO)Arquivos de Neuro-Psiquiatria1678-42272010-12-0168684985410.1590/S0004-282X2010000600004S0004-282X2010000600004Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 casesPaulo J. Lorenzoni0Rosana H. Scola1Cláudia S. Kamoi Kay2Sérgio F. Parolin3Lineu C. Werneck4Universidade Federal do ParanáUniversidade Federal do ParanáUniversidade Federal do ParanáUniversidade Federal do ParanáUniversidade Federal do ParanáLambert-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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000600004&lng=en&tlng=ensíndrome miastênica de Lambert-Eatonsíndrome miastênicaanticorpo anti-canal de cálcio voltagem dependenteestimulação nervosa repetitivaestudo eletrofisiológicotratamento |
| spellingShingle | Paulo J. Lorenzoni Rosana H. Scola Cláudia S. Kamoi Kay Sérgio F. Parolin Lineu C. Werneck Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases Arquivos de Neuro-Psiquiatria síndrome miastênica de Lambert-Eaton síndrome miastênica anticorpo anti-canal de cálcio voltagem dependente estimulação nervosa repetitiva estudo eletrofisiológico tratamento |
| title | Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases |
| title_full | Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases |
| title_fullStr | Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases |
| title_full_unstemmed | Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases |
| title_short | Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases |
| title_sort | non paraneoplastic lambert eaton myasthenic syndrome a brief review of 10 cases |
| topic | síndrome miastênica de Lambert-Eaton síndrome miastênica anticorpo anti-canal de cálcio voltagem dependente estimulação nervosa repetitiva estudo eletrofisiológico tratamento |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000600004&lng=en&tlng=en |
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