Lennox-Gastaut syndrome

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Lennox-Gastaut syndrome

ORPHA:2382Disease

What it is

A rare, severe early-onset developmental epileptic encephalopathy characterized by the triad of intellectual impairment, multiple seizure types, and typical electroencephalography (EEG) abnormalities.

Key facts

Prevalence
1-5 / 10 000 (Europe)
Age of onset
Childhood, Infancy
Inheritance
Autosomal dominant, Multigenic/multifactorial, Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

CACNA1ADisease-causing germline mutation(s) (loss of function)
CHD2Disease-causing germline mutation(s)
CUX2Disease-causing germline mutation(s)
DNM1Disease-causing germline mutation(s)
GABRB3Disease-causing germline mutation(s)
SCN1ADisease-causing germline mutation(s)
MAPK10Candidate gene tested

ICD-10 codes

G40.4filed under a broader ICD-10 category — shared with 28 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Benefit programs to look at

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

GARD 9912MEDDRA 10048816MESH D065768MONDO 0016532OMIM 615369OMIM 616346OMIM 617113OMIM 618141UMLS C0238111

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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