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Lambert–Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer
- Source :
- Journal of Neurology. 264:1854-1863
- Publication Year :
- 2017
- Publisher :
- Springer Science and Business Media LLC, 2017.
-
Abstract
- Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms. Some LEMS clinical symptoms overlap with those of other myasthenic syndromes, most commonly myasthenia gravis, which can contribute to misdiagnosis or delayed diagnosis. Prognosis is related to the presence of cancer or autoimmune disease and the severity/distribution of muscle weakness. Cause of death in patients with SCLC-LEMS is typically tumor progression, whereas NT-LEMS does not reduce life expectancy. LEMS diagnosis is supported by a threefold approach: clinical features, electromyography, and anti-VGCC antibody serology. LEMS is a clinically important early indicator of possible cancer; therefore, a LEMS diagnosis should immediately prompt rigorous oncological screening and surveillance. Symptomatic treatment of LEMS typically involves medications that improve neurotransmission (e.g., the potassium channel blocker amifampridine [3,4-diaminopyridine]), with addition of immunosuppressants/modulators (e.g., prednisone plus azathioprine) in individuals with persistent symptoms. Where a tumor is identified, oncological treatment should take priority. It should be remembered, however, that LEMS has a significant impact on a patient's quality of life and ability to perform daily activities, and therefore warrants timely diagnosis and appropriate treatment in and of itself.
- Subjects :
- Pediatrics
medicine.medical_specialty
Lung Neoplasms
Neurology
Azathioprine
03 medical and health sciences
0302 clinical medicine
Risk Factors
Prednisone
medicine
Humans
Autoimmune disease
business.industry
Muscle weakness
medicine.disease
Small Cell Lung Carcinoma
Myasthenia gravis
Lambert-Eaton Myasthenic Syndrome
030220 oncology & carcinogenesis
Immunology
Quality of Life
Etiology
Calcium Channels
Neurology (clinical)
medicine.symptom
business
Lambert-Eaton myasthenic syndrome
030217 neurology & neurosurgery
medicine.drug
Subjects
Details
- ISSN :
- 14321459 and 03405354
- Volume :
- 264
- Database :
- OpenAIRE
- Journal :
- Journal of Neurology
- Accession number :
- edsair.doi.dedup.....d7c165d122287b6449e7bb339ff7a8d0