Lennox-Gastaut syndrome
MONDO:0016532Mondo
Findings
No curated finding names Lennox-Gastaut syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Lennox-Gastaut syndrome (LGS) belongs to the group of severe childhood epileptic encephalopathies.
Definition from the Mondo Disease Ontology (MONDO:0016532), read 2026-09-29. CC BY 4.0.
Features
25 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- EEG with focal sharp slow wavesHPOHP:0011195
- Very frequent (80% to 99% of cases)
- EncephalopathyHPOHP:0001298
- Very frequent (80% to 99% of cases)
- Intellectual disabilityHPOHP:0001249
- Very frequent (80% to 99% of cases)
- Abnormal brainstem morphologyHPOHP:0002363
- Frequent (30% to 79% of cases)
- Aggressive behaviorHPOHP:0000718
- Frequent (30% to 79% of cases)
- Atonic seizureHPOHP:0010819
- Frequent (30% to 79% of cases)
- Atypical absence seizureHPOHP:0007270
- Frequent (30% to 79% of cases)
- Atypical behaviorHPOHP:0000708
- Frequent (30% to 79% of cases)
- Autistic behaviorHPOHP:0000729
- Frequent (30% to 79% of cases)
- Bilateral tonic-clonic seizureHPOHP:0002069
- Frequent (30% to 79% of cases)
- EEG abnormalityHPOHP:0002353
- Frequent (30% to 79% of cases)
- FallsHPOHP:0002527
- Frequent (30% to 79% of cases)
Show the remaining 13
- Generalized tonic seizureHPOHP:0010818
- Frequent (30% to 79% of cases)
- HyperactivityHPOHP:0000752
- Frequent (30% to 79% of cases)
- IrritabilityHPOHP:0000737
- Frequent (30% to 79% of cases)
- Mental deteriorationHPOHP:0001268
- Frequent (30% to 79% of cases)
- MyoclonusHPOHP:0001336
- Frequent (30% to 79% of cases)
- Neurodevelopmental delayHPOHP:0012758
- Frequent (30% to 79% of cases)
Genes
7 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- CACNA1AHGNC:1388
- Supportive · Orphanet · Autosomal dominant · 2021
- CHD2HGNC:1917
- Supportive · Orphanet · Autosomal dominant · 2021
- CUX2HGNC:19347
- Supportive · Orphanet · Autosomal dominant · 2021
- DNM1HGNC:2972
- Supportive · Orphanet · Autosomal dominant · 2021
- GABRB3HGNC:4083
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
1 name
Resolves to: Lennox-Gastaut syndrome
- Also called
- LGS