ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Emergency: requires immediate attention
Lennox-Gastaut syndrome
Print
Other Resources UpToDate PubMed
Emergency: requires immediate attention

Lennox-Gastaut syndrome

Print Images (1)
Contributors: Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Lennox-Gastaut syndrome is a severe epilepsy syndrome characterized by frequent seizures, intellectual impairment, and slow spike and wave activity on interictal electroencephalogram (EEG). About 70% of cases are due to underlying pathology such as hypoxic ischemic injury or other birth injuries, brain malformations, metabolic disorders, tuberous sclerosis, meningitis, or encephalitis. Some cases are idiopathic with no identifiable cause. There may be many types of seizures including tonic, myoclonic, atonic, tonic-clonic, and atypical absence. Seizures are often difficult to treat. Slowed psychomotor development, intellectual disability, and behavior problems are also common.

Typical age of onset is 2-6 years old. Patients may have progressed from prior diagnoses of infantile spasms (West syndrome). Prognosis is variable, although unfavorable overall, with many patients suffering from intractable seizures, intellectual disability, and psychiatric disorders.

Codes

ICD10CM:
G40.812 – Lennox-Gastaut syndrome, not intractable, without status epilepticus

SNOMEDCT:
230418006 – Lennox-Gastaut Syndrome

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Juvenile myoclonic epilepsy
  • Severe myoclonic epilepsy in infancy (Dravet syndrome)
  • Landau-Kleffner syndrome
  • Early infantile epileptic encephalopathy (Ohtahara syndrome)
  • Migrating partial seizures in infancy
  • Myoclonic astatic epilepsy (Doose syndrome)
  • Myoclonic status in nonprogressive encephalopathies
  • Myoclonus epilepsy with ragged red fibers (MERRF syndrome)
  • Neuronal ceroid lipofuscinoses
  • Unverricht-Lundborg disease
  • Sialidosis
  • Gaucher disease
  • Niemann-Pick disease
  • Tuberous sclerosis
  • Rett syndrome

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 09/06/2018
Copyright © 2019 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Lennox-Gastaut syndrome
Print 1 Images
Lennox-Gastaut syndrome : Seizures, Recurring episodes or relapses, Developmental delay
Copyright © 2019 VisualDx®. All rights reserved.