Eaton-Lambert syndrome is an autoimmune disorder that impairs communication between nerves and muscles, causing weakness.
- Eaton-Lambert syndrome usually precedes, occurs with, or develops after certain cancers, especially lung cancer in men.
- This syndrome causes muscle weakness (particularly in the legs), fatigue, dry mouth, drooping eyelids, and pain in the upper arms and thighs.
- Doctors suspect Eaton-Lambert syndrome based on symptoms, but electromyography is necessary for diagnosis.
- Treatment of the cancer, if present, sometimes relieves symptoms, as can guanidine, and corticosteroids and plasma exchange help some people.
(See also Overview of Neuromuscular Junction Disorders.)
Nerves communicate with muscles by releasing a chemical messenger (neurotransmitter), which interacts with receptors on muscles (at the neuromuscular junction) and stimulates muscles to contract. Eaton-Lambert syndrome is caused by antibodies that interfere with the release of the neurotransmitter acetylcholine rather than attack acetylcholine receptors (as occurs in myasthenia gravis).
Eaton-Lambert syndrome usually precedes, occurs with, or develops after certain cancers—for example, as a paraneoplastic syndrome. Paraneoplastic syndromes result from substances produced by the cancer or by the immune system in response to the cancer. Eaton-Lambert syndrome most commonly occurs in men with tumors in their chest, especially lung cancer.
Symptoms of Eaton-Lambert Syndrome
Eaton-Lambert syndrome causes muscle weakness that tends to begin in the hip and thigh muscles, then typically spreads to the shoulder muscles, and then down the arms and legs to the hands and feet. The nerves that connect the head, face, eyes, nose, muscles, and ears to the brain (cranial nerves) are affected last.
Typically, people have difficulty getting up from a chair, climbing stairs, and walking. Muscle strength may temporarily improve after the muscles are used repeatedly, but the muscles then weaken again and cramp. People also tire easily.
The mouth is dry, the eyelid droops, and the upper arms and thighs are painful.
Men may have erectile dysfunction.
Diagnosis of Eaton-Lambert Syndrome
Doctors suspect Eaton-Lambert syndrome based on symptoms, but electromyography (inserting a needle into a muscle to record its electrical activity) is needed to confirm the diagnosis.
Treatment of Eaton-Lambert Syndrome
- Treatment of cancer if present
- Sometimes corticosteroids and plasma exchange
Treating cancer, if present, sometimes relieves symptoms due to Eaton-Lambert syndrome.
Guanidine, a drug that increases the release of acetylcholine, often lessens symptoms but may inhibit the bone marrow’s production of blood cells and impair liver function.
Corticosteroids and plasma exchange (filtering of toxic substances, including abnormal antibodies, from the blood) help some people.
Drugs Mentioned In This Article
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|guanidine||No US brand name|