Disease Directory Lambert-Eaton Myasthenic Syndrome
Neuromuscular

Lambert-Eaton Myasthenic Syndrome

Also known as: LEMS, Lambert-Eaton syndrome, VGCC antibody disease

Prevalence

1 in 100,000

Onset

Adulthood; paraneoplastic form typically in 6th decade

Type

Acquired autoimmune (paraneoplastic or autoimmune)

About Lambert-Eaton Myasthenic Syndrome

Lambert-Eaton Myasthenic Syndrome is an autoimmune disorder of the neuromuscular junction caused by antibodies against voltage-gated calcium channels (VGCC) on presynaptic motor nerve terminals, impairing acetylcholine release. Approximately 50–60% of cases are paraneoplastic, most commonly associated with small cell lung cancer (SCLC). The hallmark electrodiagnostic feature is facilitation of the compound muscle action potential (CMAP) at high-frequency repetitive nerve stimulation.

Common Clinical Features

Proximal limb weakness (lower limbs predominant) Paradoxical transient strength improvement after brief exertion Autonomic dysfunction (dry mouth, constipation, erectile dysfunction) Reduced or absent deep tendon reflexes that partially return after exercise Ptosis and diplopia (less prominent than in myasthenia gravis) Dysphagia and dysarthria Fatigue worsening with sustained activity

Clinical Trial Eligibility Tips

What to know before applying to Lambert-Eaton Myasthenic Syndrome trials.

Anti-VGCC antibody seropositivity is a key eligibility criterion for immunotherapy trials; ensure serum antibody levels are quantified at a reference neurology laboratory before applying

Paraneoplastic LEMS requires cancer screening (chest CT and onconeural antibody panel) before trial enrolment; active cancer may be an exclusion criterion for non-oncology trials

Quantitative Myasthenia Gravis (QMG) score and LEMS Clinical Score (LCS) are standard endpoints; baseline scores should be documented by a neuromuscular specialist

Patient Resources

Patient Organization

Myasthenia Gravis Foundation of America

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Orphanet

European reference resource for rare diseases (ORPHA:43)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Lambert-Eaton Myasthenic Syndrome trials

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