Lambert-Eaton myasthenic syndrome.
The Lambert-Eaton myasthenic syndrome is a neuromuscular disorder characterised by defective neurotransmitter release at autonomic neurones and presynaptic terminals of the neuromuscular junction. It is caused by an IgG autoantibody formed against especially the P/Q type of voltage-gated calcium channels (VGCC) which is an essential component of the mechanism of neurotransmitter release. Many patients have an associated small cell carcinoma of the lung which appears to provide the antigenic stimulus for antibody production, although there is another group with no underlying malignancy. Both groups show an association with immunological disorders. Assay of VGCC antibody titres and electrophysiological tests help to differentiate Lambert-Eaton myasthenic syndrome from other disorders of the neuromuscular junction. Several drugs and therapeutic interventions capable of producing significant clinical improvement are currently available. Patients should also be investigated for underlying tumours, the specific treatment of which can result in remission or amelioration of symptoms.
Seneviratne
U
U
Department of Neurology, Southern General Hospital, Glasgow, UK.
de Silva
R
R
D015761:4-Aminopyridine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000077770:Amifampridine; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D015220:Calcium Channels / Q000276:immunology / Q000378:metabolism; D018288:Carcinoma, Small Cell / Q000150:complications; D020022:Genetic Predisposition to Disease; D015795:HLA-B8 Antigen; D006801:Humans; D016756:Immunoglobulins, Intravenous; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D008175:Lung Neoplasms / Q000150:complications; D010951:Plasma Exchange; D015221:Potassium Channels / Q000187:drug effects; D011239:Prednisolone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D015220:Calcium Channels; D015795:HLA-B8 Antigen; D016756:Immunoglobulins, Intravenous; D015221:Potassium Channels; D011239:Prednisolone; D015761:4-Aminopyridine; D000077770:Amifampridine