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Lambert Eaton Myasthenic Syndrome - Symptoms and Treatment
Lambert-Eaton syndrome is characterized by muscle weakness that improves with continued contraction of the muscle . It is caused by a disruption of electrical impulses between these nerve and muscle cells. Lambert-Eaton myasthenic syndrome is an autoimmune condition; in such disorders the immune system, which normally protects the body from foreign organisms, mistakenly attacks the body's own tissues. The disruption of electrical impulses is associated with antibodies produced as a consequence of this autoimmunity.
Symptoms include muscle weakness, a tingling sensation in the affected areas, fatigue, and dry mouth. Lambert Eaton Myasthenic Syndrome is less than 1 in every 1,000,000 people. The disorder is not hereditary. LEMS develops when antibodies are produced that prevent the neurotransmitter acetylcholine from being released from nerve endings joined to muscles. In the absence of acetylcholine release, muscle cannot contract, producing weakness. LEMS antibodies can develop in otherwise healthy persons, or can sometimes develop in the presence of lung cancer.
Lambert-Eaton myasthenic syndrome is a disorder of the neuromuscular junction-the site where nerve cells meet muscle cells and help activate the muscles. Both the etiology and the clinical findings of the disease may resemble myasthenia gravis , but there are many substantial differences between clinical presentations and pathogenetic features of two disorders.
Lambert-Eaton myasthenic syndrome is associated in 40% of cases with cancer, most often with small cell cancer of the lung and less often with other tumors. The neuromuscular defect in LEMS is due to insufficient release of the neurotransmitter acetylcholine by nerve cells. LEMS has been treated with pyridostigmine bromide to increase the transmission of acetylcholine across the neuromuscular junction, a drug called diaminopyridine and immunosuppressants (the steroid prednisone, azathioprine, cyclosporine). Plasma exchange provides improvement in some patients with LEMS, as may intravenous immunoglobulin (IVIg). Patients over 50 with a history of long-term smoking are most likely to have an associated tumor.
Causes of Lambert Eaton Myasthenic Syndrome
Lambert-Eaton myasthenic syndrome is an uncommon neuromuscular disorder characterized by weakness in muscles of the upper arms and upper legs, and less commonly, muscles of the neck, speech, swallowing, breathing and eye movement. In myasthenia gravis, the neurotransmitter acetylcholine is blocked by antibodies to its receptor. In Lambert-Eaton syndrome, however, the signal distortion is caused by an insufficient release of neurotransmitter by the nerve cell. As muscle contraction is continued, the amount of neurotransmitter may build up in sufficient quantities and result in increased strength. The disorder may be associated with small-cell carcinoma of the lung and other malignancies as a paraneoplastic syndrome or with autoimmune disorders .
Signs and Symptoms of Lambert Eaton Myasthenic Syndrome
Sign and symptoms may include the following :
Treatment for Lambert Eaton Myasthenic Syndrome
There is no cure for Lambert-Eaton myasthenic syndrome . However, multiple treatment options are available to improve symptoms and sometimes produce remission. Symptomatic treatment consists of 3,4-diaminopyridine (3,4-DAP). LEMS has been treated with pyridostigmine bromide (Mestinon) to increase the transmission of acetylcholine across the neuromuscular junction, a drug called diaminopyridine (DAP) and immunosuppressants (the steroid prednisone, azathioprine, cyclosporine). Plasma exchange provides improvement in some patients with LEMS, as may intravenous immunoglobulin (IVIg). Patients over 50 with a history of long-term smoking are most likely to have an associated tumor. If the tumor is cured, the LEMS may vanish.
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