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Lennox-Gastaut syndrome

MedGen UID:
116044
•Concept ID:
C0238111
•
Disease or Syndrome
Synonym: Epileptic encephalopathy Lennox-Gastaut type
SNOMED CT: Lennox-Gastaut syndrome (230418006)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Autosomal dominant inheritance (Orphanet)
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016532
Orphanet: ORPHA2382

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLennox-Gastaut syndrome
Follow this link to review classifications for Lennox-Gastaut syndrome in Orphanet.

Professional guidelines

PubMed

Epilepsy: Epileptic Syndromes and Treatment.
Katyayan A, Diaz-Medina G
Neurol Clin 2021 Aug;39(3):779-795. doi: 10.1016/j.ncl.2021.04.002. PMID: 34215386
Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies.
Strzelczyk A, Schubert-Bast S
CNS Drugs 2021 Jan;35(1):61-83. Epub 2021 Jan 21 doi: 10.1007/s40263-020-00784-8. PMID: 33479851Free PMC Article
Epilepsy and cannabidiol: a guide to treatment.
Arzimanoglou A, Brandl U, Cross JH, Gil-Nagel A, Lagae L, Landmark CJ, Specchio N, Nabbout R, Thiele EA, Gubbay O, The Cannabinoids International Experts Panel; Collaborators
Epileptic Disord 2020 Feb 1;22(1):1-14. doi: 10.1684/epd.2020.1141. PMID: 32096470

Recent clinical studies

Etiology

Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial.
Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Cannabidiol in the Treatment of Epilepsy.
von Wrede R, Helmstaedter C, Surges R
Clin Drug Investig 2021 Mar;41(3):211-220. Epub 2021 Feb 9 doi: 10.1007/s40261-021-01003-y. PMID: 33559102Free PMC Article
Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies.
Strzelczyk A, Schubert-Bast S
CNS Drugs 2021 Jan;35(1):61-83. Epub 2021 Jan 21 doi: 10.1007/s40263-020-00784-8. PMID: 33479851Free PMC Article
Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome.
Devinsky O, Patel AD, Cross JH, Villanueva V, Wirrell EC, Privitera M, Greenwood SM, Roberts C, Checketts D, VanLandingham KE, Zuberi SM; GWPCARE3 Study Group
N Engl J Med 2018 May 17;378(20):1888-1897. doi: 10.1056/NEJMoa1714631. PMID: 29768152
Lennox-Gastaut syndrome: a comprehensive review.
Asadi-Pooya AA
Neurol Sci 2018 Mar;39(3):403-414. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3188-y. PMID: 29124439

Diagnosis

Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial.
Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Epilepsy: Epileptic Syndromes and Treatment.
Katyayan A, Diaz-Medina G
Neurol Clin 2021 Aug;39(3):779-795. doi: 10.1016/j.ncl.2021.04.002. PMID: 34215386
Expert opinion: Proposed diagnostic and treatment algorithms for Lennox-Gastaut syndrome in adult patients.
Montouris G, Aboumatar S, Burdette D, Kothare S, Kuzniecky R, Rosenfeld W, Chung S
Epilepsy Behav 2020 Sep;110:107146. Epub 2020 Jun 18 doi: 10.1016/j.yebeh.2020.107146. PMID: 32563898
Lennox-Gastaut syndrome: a comprehensive review.
Asadi-Pooya AA
Neurol Sci 2018 Mar;39(3):403-414. Epub 2017 Nov 9 doi: 10.1007/s10072-017-3188-y. PMID: 29124439
Seizures and epilepsy: an overview for neuroscientists.
Stafstrom CE, Carmant L
Cold Spring Harb Perspect Med 2015 Jun 1;5(6) doi: 10.1101/cshperspect.a022426. PMID: 26033084Free PMC Article

Therapy

Recent advances in pharmacotherapy for epilepsy.
Pong AW, Xu KJ, Klein P
Curr Opin Neurol 2023 Apr 1;36(2):77-85. Epub 2023 Feb 10 doi: 10.1097/WCO.0000000000001144. PMID: 36762638
Cannabidiol in the Treatment of Epilepsy.
von Wrede R, Helmstaedter C, Surges R
Clin Drug Investig 2021 Mar;41(3):211-220. Epub 2021 Feb 9 doi: 10.1007/s40261-021-01003-y. PMID: 33559102Free PMC Article
Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies.
Strzelczyk A, Schubert-Bast S
CNS Drugs 2021 Jan;35(1):61-83. Epub 2021 Jan 21 doi: 10.1007/s40263-020-00784-8. PMID: 33479851Free PMC Article
Cannabidiol Therapy for Refractory Epilepsy and Seizure Disorders.
Golub V, Reddy DS
Adv Exp Med Biol 2021;1264:93-110. doi: 10.1007/978-3-030-57369-0_7. PMID: 33332006
Epilepsy and cannabidiol: a guide to treatment.
Arzimanoglou A, Brandl U, Cross JH, Gil-Nagel A, Lagae L, Landmark CJ, Specchio N, Nabbout R, Thiele EA, Gubbay O, The Cannabinoids International Experts Panel; Collaborators
Epileptic Disord 2020 Feb 1;22(1):1-14. doi: 10.1684/epd.2020.1141. PMID: 32096470

Prognosis

Epileptogenesis in tuberous sclerosis complex-related developmental and epileptic encephalopathy.
Aronica E, Specchio N, Luinenburg MJ, Curatolo P
Brain 2023 Jul 3;146(7):2694-2710. doi: 10.1093/brain/awad048. PMID: 36806388Free PMC Article
International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions.
Specchio N, Wirrell EC, Scheffer IE, Nabbout R, Riney K, Samia P, Guerreiro M, Gwer S, Zuberi SM, Wilmshurst JM, Yozawitz E, Pressler R, Hirsch E, Wiebe S, Cross HJ, Perucca E, Moshé SL, Tinuper P, Auvin S
Epilepsia 2022 Jun;63(6):1398-1442. Epub 2022 May 3 doi: 10.1111/epi.17241. PMID: 35503717
Epilepsy: Epileptic Syndromes and Treatment.
Katyayan A, Diaz-Medina G
Neurol Clin 2021 Aug;39(3):779-795. doi: 10.1016/j.ncl.2021.04.002. PMID: 34215386
Surgery for epilepsy.
West S, Nevitt SJ, Cotton J, Gandhi S, Weston J, Sudan A, Ramirez R, Newton R
Cochrane Database Syst Rev 2019 Jun 25;6(6):CD010541. doi: 10.1002/14651858.CD010541.pub3. PMID: 31237346Free PMC Article
Seizures and epilepsy: an overview for neuroscientists.
Stafstrom CE, Carmant L
Cold Spring Harb Perspect Med 2015 Jun 1;5(6) doi: 10.1101/cshperspect.a022426. PMID: 26033084Free PMC Article

Clinical prediction guides

Fenfluramine: A Review in Dravet and Lennox-Gastaut Syndromes.
Frampton JE
Drugs 2023 Jul;83(10):923-934. Epub 2023 Jun 15 doi: 10.1007/s40265-023-01881-w. PMID: 37316680Free PMC Article
Efficacy and Safety of Fenfluramine for the Treatment of Seizures Associated With Lennox-Gastaut Syndrome: A Randomized Clinical Trial.
Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Highly Purified Cannabidiol for Epilepsy Treatment: A Systematic Review of Epileptic Conditions Beyond Dravet Syndrome and Lennox-Gastaut Syndrome.
Lattanzi S, Trinka E, Striano P, Rocchi C, Salvemini S, Silvestrini M, Brigo F
CNS Drugs 2021 Mar;35(3):265-281. Epub 2021 Mar 22 doi: 10.1007/s40263-021-00807-y. PMID: 33754312Free PMC Article
Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies.
Strzelczyk A, Schubert-Bast S
CNS Drugs 2021 Jan;35(1):61-83. Epub 2021 Jan 21 doi: 10.1007/s40263-020-00784-8. PMID: 33479851Free PMC Article
De novo mutations in epileptic encephalopathies.
Epi4K Consortium; Epilepsy Phenome/Genome Project, Allen AS, Berkovic SF, Cossette P, Delanty N, Dlugos D, Eichler EE, Epstein MP, Glauser T, Goldstein DB, Han Y, Heinzen EL, Hitomi Y, Howell KB, Johnson MR, Kuzniecky R, Lowenstein DH, Lu YF, Madou MR, Marson AG, Mefford HC, Esmaeeli Nieh S, O'Brien TJ, Ottman R, Petrovski S, Poduri A, Ruzzo EK, Scheffer IE, Sherr EH, Yuskaitis CJ, Abou-Khalil B, Alldredge BK, Bautista JF, Berkovic SF, Boro A, Cascino GD, Consalvo D, Crumrine P, Devinsky O, Dlugos D, Epstein MP, Fiol M, Fountain NB, French J, Friedman D, Geller EB, Glauser T, Glynn S, Haut SR, Hayward J, Helmers SL, Joshi S, Kanner A, Kirsch HE, Knowlton RC, Kossoff EH, Kuperman R, Kuzniecky R, Lowenstein DH, McGuire SM, Motika PV, Novotny EJ, Ottman R, Paolicchi JM, Parent JM, Park K, Poduri A, Scheffer IE, Shellhaas RA, Sherr EH, Shih JJ, Singh R, Sirven J, Smith MC, Sullivan J, Lin Thio L, Venkat A, Vining EP, Von Allmen GK, Weisenberg JL, Widdess-Walsh P, Winawer MR
Nature 2013 Sep 12;501(7466):217-21. Epub 2013 Aug 11 doi: 10.1038/nature12439. PMID: 23934111Free PMC Article

Recent systematic reviews

The burden of illness in Lennox-Gastaut syndrome: a systematic literature review.
Strzelczyk A, Zuberi SM, Striano P, Rosenow F, Schubert-Bast S
Orphanet J Rare Dis 2023 Mar 1;18(1):42. doi: 10.1186/s13023-023-02626-4. PMID: 36859290Free PMC Article
Clinical efficacy and safety of cannabidiol for pediatric refractory epilepsy indications: A systematic review and meta-analysis.
Talwar A, Estes E, Aparasu R, Reddy DS
Exp Neurol 2023 Jan;359:114238. Epub 2022 Oct 4 doi: 10.1016/j.expneurol.2022.114238. PMID: 36206805
Highly Purified Cannabidiol for Epilepsy Treatment: A Systematic Review of Epileptic Conditions Beyond Dravet Syndrome and Lennox-Gastaut Syndrome.
Lattanzi S, Trinka E, Striano P, Rocchi C, Salvemini S, Silvestrini M, Brigo F
CNS Drugs 2021 Mar;35(3):265-281. Epub 2021 Mar 22 doi: 10.1007/s40263-021-00807-y. PMID: 33754312Free PMC Article
Surgery for epilepsy.
West S, Nevitt SJ, Cotton J, Gandhi S, Weston J, Sudan A, Ramirez R, Newton R
Cochrane Database Syst Rev 2019 Jun 25;6(6):CD010541. doi: 10.1002/14651858.CD010541.pub3. PMID: 31237346Free PMC Article
Efficacy and Safety of Cannabidiol in Epilepsy: A Systematic Review and Meta-Analysis.
Lattanzi S, Brigo F, Trinka E, Zaccara G, Cagnetti C, Del Giovane C, Silvestrini M
Drugs 2018 Nov;78(17):1791-1804. doi: 10.1007/s40265-018-0992-5. PMID: 30390221

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