Lambert–Eaton myasthenic syndrome
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Lambert–Eaton myasthenic syndrome | |
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Synonyms | N/A |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Muscle weakness, fatigue, dry mouth, erectile dysfunction, constipation |
Complications | N/A |
Onset | Typically in adulthood |
Duration | Chronic |
Types | N/A |
Causes | Autoimmune disease, paraneoplastic syndrome |
Risks | Small cell lung cancer, other cancers |
Diagnosis | Clinical diagnosis, electromyography, antibody testing |
Differential diagnosis | Myasthenia gravis, botulism, Guillain–Barré syndrome |
Prevention | N/A |
Treatment | 3,4-diaminopyridine, pyridostigmine, immunosuppressive drugs |
Medication | N/A |
Prognosis | Variable, depends on underlying cause |
Frequency | Rare |
Deaths | N/A |
Lambert–Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder that affects the neuromuscular junction, the site where nerve cells communicate with muscles. It is characterized by muscle weakness and fatigue, particularly in the proximal muscles, which are the muscles closest to the trunk of the body.
Pathophysiology[edit | edit source]
LEMS is caused by autoantibodies that target voltage-gated calcium channels (VGCCs) on the presynaptic nerve terminals. These autoantibodies inhibit the release of acetylcholine, a neurotransmitter essential for muscle contraction. The reduction in acetylcholine release leads to the characteristic muscle weakness seen in LEMS.
Symptoms[edit | edit source]
The primary symptoms of LEMS include:
- Muscle weakness, especially in the upper legs and hips
- Fatigue
- Difficulty climbing stairs or rising from a seated position
- Dry mouth
- Erectile dysfunction in men
- Autonomic dysfunction, such as constipation and blurred vision
Diagnosis[edit | edit source]
The diagnosis of LEMS is typically based on clinical evaluation, electromyography (EMG), and the detection of anti-VGCC antibodies in the blood. EMG studies often show a characteristic incremental response to repetitive nerve stimulation.
Treatment[edit | edit source]
Treatment for LEMS may include:
- Medications to enhance acetylcholine release, such as 3,4-diaminopyridine (3,4-DAP)
- Immunosuppressive therapies, such as corticosteroids and azathioprine
- Plasmapheresis or intravenous immunoglobulin (IVIG) to reduce the levels of autoantibodies
Prognosis[edit | edit source]
The prognosis for individuals with LEMS varies. Some patients respond well to treatment and experience significant improvement in muscle strength and function. However, LEMS is often associated with underlying malignancies, particularly small cell lung cancer, which can affect the overall prognosis.
Related Conditions[edit | edit source]
LEMS is often associated with other autoimmune disorders and malignancies. It is important to screen for underlying cancers, especially in older patients and smokers.
See Also[edit | edit source]
References[edit | edit source]
External Links[edit | edit source]
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Contributors: Prab R. Tumpati, MD