Lambert-Eaton syndrome (Lambert-Eaton myasthenic syndrome)
What is it?
Eaton-Lambert myasthenic syndrome (LEMS) is a rare disorder that affects the neuromuscular junction, the structure that allows electrical impulses from nerves to be transmitted to the muscles. It is an autoimmune disease in which damage is caused by antibodies that our immune system makes against the neuromuscular junction. It is an acquired disease (humans are not born with the disease). In 50-60% of cases, this is associated with a tumor, most often of the lung.
Which are the symptoms?
Onset usually occurs in adulthood. The main symptom is weakness, which mainly affects the proximal muscles of the extremities (thighs and buttocks, less often the shoulders), but in the future it can affect all muscles. Symptoms such as dry mouth, erectile dysfunction, and constipation (called autonomic symptoms) are also common with weakness. Electromyography and the search for antibodies against calcium channels are essential for diagnosis.
How is it diagnosed?
After the diagnosis is made, tests (in particular CT and PET-CT) should be performed to rule out the presence of a tumor; in case of a negative result, the check must be repeated periodically in the coming months.
Suggested exams
How is it treated?
LEMS weakness responds well to 3,4-diaminopyridine treatment. If a neoplasm is found, treatment usually involves recovery. Resistant forms can respond to immunosuppressive therapy or intravenous immunoglobulins.
Where do we treat it?
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