Lennox-Gastaut syndrome (epileptic encephalopathy, Lennox-Gastaut type)
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Introduction
Generalized myoclonic epilepsy in children.
Etiology
- perinatal hypoxia
- cerebral hemorrhage
- encephalitis
- developmental disorders of the brain
- 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
- mental retardation
- multiple seizure types
- developmentally delayed
Laboratory
- electroencephalogram (EEG)
- background slowing
- slow spike & wave pattern
Management
- anticonvulsants to control seizures
- seizures are generally resistant to most anticonvulsants
- cannabidiol 20 mg/kg daily decreases seizures 40%[2]
- prognosis is variable
- no cure
- cessation of seizures & normal development is rare
More general terms
Additional terms
References
- ↑ Wikipedia: Lennox-Gastaut syndrome https://en.wikipedia.org/wiki/Lennox%E2%80%93Gastaut_syndrome
- ↑ 2.0 2.1 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 - ↑ 3.0 3.1 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: https://www.ncbi.nlm.nih.gov/pubmed/29768152 https://www.nejm.org/doi/full/10.1056/NEJMoa1714631
- ↑ NINDS Lennox-Gastaut Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Lennox-Gastaut-Syndrome-Information-Page
Patient information
Lennox-Gastaut syndrome patient information