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Atlas / Disease / Lambert-Eaton myasthenic syndrome

Lambert-Eaton myasthenic syndrome

disease
On this page

Also known as Eaton Lambert syndromeEaton-Lambert syndromeLambert Eaton myasthenic syndromeLambert Eaton syndromeLambert-Eaton syndromemyasthenic syndrome of Lambert-Eatonmyasthenic-myopathic syndrome of Lambert-Eaton

Summary

Lambert-Eaton myasthenic syndrome (MONDO:0018556) is a disease and 18 clinical trials. Top therapeutic interventions include amifampridine. A subtype of paraneoplastic neurologic syndrome — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: 1-9 / 1 000 000 (Worldwide) [Orphanet-validated]
  • Phenotypes (HPO): 16
  • Clinical trials: 18

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Point prevalence1-9 / 1 000 0000.35WorldwideValidated
Point prevalence1-9 / 100 0001EuropeValidated
Annual incidence<1 / 1 000 0000.06NetherlandsValidated

Signs & symptoms

Clinical features (HPO)

16 HPO clinical features (Orphanet curated; top 16 by frequency):

HPO IDTermFrequency
HP:0000217XerostomiaVery frequent (80-99%)
HP:0001315Reduced tendon reflexesVery frequent (80-99%)
HP:0003403EMG: decremental response of compound muscle action potential to repetitive nerve stimulationVery frequent (80-99%)
HP:0009073Progressive proximal muscle weaknessVery frequent (80-99%)
HP:0012332Abnormal autonomic nervous system physiologyVery frequent (80-99%)
HP:0030000EMG: repetitive nerve stimulation abnormalityVery frequent (80-99%)
HP:0030209Calcium channel antibody positivityVery frequent (80-99%)
HP:0000315Abnormality of the orbital regionFrequent (30-79%)
HP:0000802ImpotenceFrequent (30-79%)
HP:0002019ConstipationFrequent (30-79%)
HP:0002483Bulbar signsFrequent (30-79%)
HP:0030357Small cell lung carcinomaFrequent (30-79%)
HP:5000027Anti-P/Q-type VGCC antibody positivityFrequent (30-79%)
HP:0000966HypohidrosisOccasional (5-29%)
HP:0001097Keratoconjunctivitis siccaOccasional (5-29%)
HP:0004926Orthostatic hypotension due to autonomic dysfunctionOccasional (5-29%)

Identifiers

Disease identifiers

FieldValue
Canonical nameLambert-Eaton myasthenic syndrome
Mondo IDMONDO:0018556
MeSHD015624
Orphanet43393
DOIDDOID:0050214
ICD-11796417193
NCITC3155
SNOMED CT56989000
UMLSC0022972
MedGen6005
GARD0006851
MedDRA10067685
NORD1346
Is cancer (heuristic)no

Also known as: Eaton Lambert syndrome · Eaton-Lambert syndrome · Lambert Eaton myasthenic syndrome · Lambert Eaton syndrome · Lambert-Eaton syndrome · myasthenic syndrome of Lambert-Eaton · myasthenic-myopathic syndrome of Lambert-Eaton

Disease family

Classification path: disease › human disease › disease by body system or component › nervous system disorder › paraneoplastic neurologic syndrome › Lambert-Eaton myasthenic syndrome

Related subtypes (6): paraneoplastic polyneuropathy, opsoclonus-myoclonus syndrome, paraneoplastic limbic encephalitis, POEMS syndrome, cancer-associated retinopathy, paraneoplastic cerebellar degeneration

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated or in trials for this disease

1 approved drug — disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugStatus
AmifampridineApproved (phase 4)

Clinical trials & evidence

Clinical trials

Clinical trials: 18.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified14
PHASE32
PHASE22

Top trials by phase / activity

NCTPhaseStatusTitle
NCT01377922PHASE3COMPLETEDA Phase 3 Study of Amifampridine Phosphate in Patients With Lambert Eaton Myasthenic Syndrome (LEMS)
NCT02970162PHASE3COMPLETEDPhase 3 Study to Evaluate Efficacy of Amifampridine Phosphate in Lambert-Eaton Myasthenic Syndrome (LEMS)
NCT00716066PHASE2ACTIVE_NOT_RECRUITINGAutologous Stem Cell Transplant for Neurologic Autoimmune Diseases
NCT01511978PHASE2COMPLETEDEffectiveness of 3,4-Diaminopyridine in Lambert-Eaton Myasthenic Syndrome
NCT06441825Not specifiedRECRUITINGPatient Observation With Environmental and Wearable Sensors in Myasthenia Gravis
NCT06605612Not specifiedENROLLING_BY_INVITATIONDevelopment and Validation of the FBIndex to Determine the Risk of Falls for Patients With Neuromuscular Disorders
NCT07075627Not specifiedNOT_YET_RECRUITINGA Study to Evaluate the Incidence of Clinically Suspicious Lambert-Eaton Myasthenic Syndrome (LEMS) in Subjects Diagnosed With Small Cell Lung Cancer (SCLC)
NCT07478172Not specifiedRECRUITINGEffects of Whole-body Electrical Muscle Stimulation Exercise on Adults With Neuromuscular Disease
NCT00004832Not specifiedCOMPLETEDRandomized Study of 3,4-Diaminopyridine for Lambert-Eaton Myasthenic Syndrome
NCT00704925Not specifiedNO_LONGER_AVAILABLETreatment of Lambert-Eaton Syndrome With 3,4 DAP
NCT00872950Not specifiedAPPROVED_FOR_MARKETING3,4-Diaminopyridine Use in Lambert-Eaton Myasthenic Syndrome(LEMS) and Congenital Myasthenic Syndromes (CMS)
NCT00994916Not specifiedNO_LONGER_AVAILABLETreatment of Lambert-Eaton Syndrome With 3,4 Diaminopyridine
NCT01373333Not specifiedNO_LONGER_AVAILABLEUse Of 3,4-Diaminopyridine (3,4-DAP) In The Treatment Of Lambert Eaton Myasthenic Syndrome
NCT01378546Not specifiedNO_LONGER_AVAILABLETreatment of Lambert-Eaton Myasthenic Syndrome (LEMS) With 3, 4 DAP
NCT01825395Not specifiedAPPROVED_FOR_MARKETINGUse of 3,4-Diaminopyridine in the Treatment of Lambert-Eaton Syndrome
NCT02012933Not specifiedNO_LONGER_AVAILABLE3,4-Diaminopyridine for Lambert-Eaton Myasthenic Syndrome (LEMS) and Congenital Myasthenia (CM)
NCT02189720Not specifiedAPPROVED_FOR_MARKETINGExpanded Access Study Amifampridine Phosphate in Lambert-Eaton Myasthenic Syndrome (LEMS),Congenital Myasthenic Syndrome
NCT05408702Not specifiedCOMPLETEDExercise in Autoimmune Myasthenia Gravis and Myasthenic Syndromes

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
AMIFAMPRIDINE45
CHEMBL1572001

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

Generated
Atlas version
v1.1.4
BioBTree
v2.0.2

Sources 24

This page pulls from 24 datasets indexed by BioBTree:

chembl_moleculecivic_evidenceclinical_trialsclinvardoidefogardgenccgwasgwas_studyhgncicd11meddramedgenmeshmimmondomondochildmondoparentncitnordorphanetsctidumls