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 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.
| Main Authors: | , , , , |
|---|---|
| Format: | Digital revista |
| Language: | English |
| Published: |
Academia Brasileira de Neurologia - ABNEURO
2010
|
| Online Access: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000600004 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|