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.
Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
Paulo J
PJ
Lorenzoni
Rosana H
RH
Scola
Cláudia S Kamoi
CS
Kay
Sérgio F
SF
Parolin
Lineu C
LC
Werneck
D000328:Adult; D004594:Electrophysiology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012189:Retrospective Studies
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents