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Lambert-Eaton Myasthenic Syndrome

Progressive muscle weakness present

By Mary Kugler, R.N., About.com

Updated: March 09, 2005

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Lambert-Eaton myasthenic syndrome is a neuromuscular disorder which causes progressive muscle weakness, usually first noticed in the upper legs and upper arms. The syndrome is due to an abnormality of the release of the chemical acetycholine, a neurotransmitter which communicates nerve impulses to muscles. Without enough acetycholine between a nerve and a muscle, the muscle doesn't receive some or all of the nerve's signal.

Research suggests that Lambert-Eaton myasthenic syndrome may be an autoimmune disorder. It usually begins in late adulthood, and the true incidence of the syndrome is not known.

Symptoms
Symptoms of Lambert-Eaton myasthenic syndrome develop slowly. They include:

  • Weakness of the upper legs and upper arms that gets worse over time
  • Dry mouth, eyes, or skin
  • Eyelid drooping or double vision (25% of individuals)

Individuals with Lambert-Eaton myasthenic syndrome who are long-term smokers are at high risk for cancer, especially small cell lung cancer. Individuals who develop the syndrome at an older age also have a greater risk of cancer. A cancerous tumor may increase the amount of muscle weakness, as well as cause fatigue and weight loss.

Diagnosis
Physical examination will provide evidence of muscle weakness of the upper legs and upper arms. For example, the individual with Lambert-Eaton may have a waddling gait and have difficulty raising his arms. A blood test for specific autoimmune antibodies can be performed. Repetitive nerve stimulation studies, which test the conduction of nerve impulses to muscles, can help confirm the diagnosis and differentiate Lambert-Eaton myasthenic syndrome from [link url=http://http://rarediseases.about.com/cs/myastheniagravis/a/071901.htm]myasthenia gravis[/link].

All individuals with Lambert-Eaton should be screened for cancer, particularly those people who are long-term smokers. A computed tomography (CT) scan of the chest, and possible a lung biopsy, are performed to look for lung tumors.

Treatment
If cancer is present, treatment should focus on cancer therapy. In some cases, the muscle weakness of Lambert-Eaton will improve as the cancer treatment progresses. In individuals with the syndrome but without cancer, immunosuppressive drugs such as prednisone and azathioprine can be used to reduce the autoimmune response and lessen symptoms.

Neuromuscular drugs can help improve muscle strength and reduce symptoms in some individuals with Lambert-Eaton. These drugs include pyridostigmine bromide (Mestinon), 3,4-diaminopyridine (DAP), and guanidine. Muscle weakness may increase with hot weather, use of hot tubs or hot showers, fever, or illness.

Information for this article was taken from:
Sanders, D. B. (2003). Lambert-Eaton myasthenic syndrome. eMedicine, accessed at http://www.emedicine.com/neuro/topic181.htm

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