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Lennox-Gastaut syndrome (epileptic encephalopathy, Lennox-Gastaut type)

Generalized myoclonic epilepsy in children. Etiology: 1) perinatal hypoxia 2) cerebral hemorrhage 3) encephalitis 4) developmental disorders of the brain 5) metabolic disorders of the brain Epidemiology: - usually develops in children between 1 and 8 years old Genetics: - chromosomal translocation t(Y;4)(q11.2;q21) involving MAPK10 is a cause of epileptic encephalopathy Lennox-Gastaut type Clinical manifestations: 1) mental retardation 2) multiple seizure types a) generalized tonic b) atonic c) myoclonic d) tonic-clonic e) atypical absence 3) developmentally delayed Laboratory: - electroencephalogram (EEG) 1) background slowing 2) slow spike & wave pattern Management: 1) anticonvulsants to control seizures 2) seizures are generally resistant to most anticonvulsants 3) cannabidiol 20 mg/kg daily decreases seizures 40% [2] - 10 mg/kg daily decreases seizures 37% with fewer adverse effects [3] 3) prognosis is variable a) no cure b) cessation of seizures & normal development is rare

Related

infantile epileptic encephalopathy

General

myoclonic epilepsy syndrome

Database Correlations

OMIM 606369

References

  1. Wikipedia: Lennox-Gastaut syndrome https://en.wikipedia.org/wiki/Lennox%E2%80%93Gastaut_syndrome
  2. Bachert A Cannabidiol Cuts Seizures in LGS Patients - Decreased appetite, sleepiness most common side effects. MedPage Today. April 18, 2017 https://www.medpagetoday.com/MeetingCoverage/AAN/64620 - Patel A, et al Cannabidiol (CBD) significantly reduces drop seizure frequency in Lennox-Gastaut syndrome (LGS): results of a dose-ranging, multi-center, randomized, double-blind, placebo-controlled trial (GWPCARE3) American Academy of Neurology (AAN) 2017
  3. Devinsky O, Patel AD, Cross JH et al Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome. N Engl J Med 2018; 378:1888-1897. May 17, 2018 PMID: 29768152 https://www.nejm.org/doi/full/10.1056/NEJMoa1714631
  4. NINDS Lennox-Gastaut Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Lennox-Gastaut-Syndrome-Information-Page