Back to Search
Start Over
[Lambert-Eaton myasthenic syndrome (LEMS)].
- Source :
-
Brain and nerve = Shinkei kenkyu no shinpo [Brain Nerve] 2010 Apr; Vol. 62 (4), pp. 419-26. - Publication Year :
- 2010
-
Abstract
- Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disorder in which autoantibodies inhibit the presynaptic release of acetylcholine. Autoantibodies against P/Q-type voltage-gated calcium channels (VGCC) are detected in 85% of patients with LEMS. In addition, autoantibodies to synaptotagmin, an M1-type muscarinic acetylcholine receptor and SOX1 are also found in the sera of patients with LEMS. LEMS is closely associated with small cell lung cancer (SCLC) in 50-60% of patients. Patients with SCLC who have anti-VGCC antibodies have been reported to have a favorable prognosis. In contrast to paraneoplatic LEMS, other forms of LEMS may have an autoimmune aspect because of the established association between human leukocyte antigen and a family history of other autoimmune disorders in this condition. The clinical features of LEMS include proximal weakness, areflexia, ptosis, cerebellar ataxia and autonomic dysfunction. The findings of electrophysiological examination show that LEMS is characterized by compound muscle action potential potentials with a low amplitude and increment upon repetitive nerve stimulation at a high rate. Tumor removal is the primary treatment of LEMS. The efficacy of 3,4-diaminopyridine for the treatment of LEMS has also been established. Patients with LEMS require the immunotherapies such as plasma exchange and the administration of high doses of immunoglobulin and prednisolone.
- Subjects :
- 4-Aminopyridine analogs & derivatives
4-Aminopyridine therapeutic use
Amifampridine
Calcium Channels, P-Type immunology
Calcium Channels, Q-Type immunology
HLA Antigens immunology
Humans
Immunotherapy
Plasma Exchange
SOXB1 Transcription Factors immunology
Synaptotagmins immunology
Autoantibodies
Lambert-Eaton Myasthenic Syndrome diagnosis
Lambert-Eaton Myasthenic Syndrome immunology
Lambert-Eaton Myasthenic Syndrome physiopathology
Lambert-Eaton Myasthenic Syndrome therapy
Paraneoplastic Syndromes, Nervous System
Subjects
Details
- Language :
- Japanese
- ISSN :
- 1881-6096
- Volume :
- 62
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Brain and nerve = Shinkei kenkyu no shinpo
- Publication Type :
- Academic Journal
- Accession number :
- 20420183