KEGG   DISEASE: Lennox-Gastaut syndrome
Entry
H01813                      Disease                                

Name
Lennox-Gastaut syndrome
  Supergrp
Early infantile epileptic encephalopathy [DS:H00606]
Symptomatic generalized epilepsies [DS:H00577]
Description
Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by multiple seizure types, typical findings in the electroencephalogram (EEG), and delayed psychomotor development. Tonic seizures during sleep are the feature often used as the foundation for diagnosis. LGS is characterized by multiple concurrent seizure types, including tonic, atypical absence seizures, atonic, and myoclonic jerks. Non-convulsive status epilepticus, lasting days to weeks, occurs in half of patients. The etiology of LGS is heterogeneous and includes both genetic and acquired causes. LGS most commonly first manifests in children between 3 and 5 years of age, but onset can also occur at younger and older ages. It has been reported that 20-36% of children diagnosed with LGS syndrome have a history of West syndrome.
Category
Nervous system disease
Brite
Human diseases [BR:br08402]
 Nervous system diseases
  Epilepsy
   H01813  Lennox-Gastaut syndrome
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Epilepsy or seizures
   8A62  Epileptic encephalopathies
    H01813  Lennox-Gastaut syndrome
Gene
MAPK10 [HSA:5602] [KO:K04440]
Drug
Clonazepam [DR:D00280]
Rufinamide [DR:D05775]
Lamotrigine [DR:D00354]
Topiramate [DR:D00537]
Clobazam [DR:D01253]
Other DBs
ICD-11: 8A62.1
ICD-10: G40.4
MeSH: D065768
OMIM: 606369
Reference
  Authors
Bourgeois BF, Douglass LM, Sankar R
  Title
Lennox-Gastaut syndrome: a consensus approach to differential diagnosis.
  Journal
Epilepsia 55 Suppl 4:4-9 (2014)
DOI:10.1111/epi.12567
Reference
  Authors
You SJ, Kim HD, Kang HC
  Title
Factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome.
  Journal
Pediatr Neurol 41:111-3 (2009)
DOI:10.1016/j.pediatrneurol.2009.03.006
Reference
  Authors
Lund C, Brodtkorb E, Oye AM, Rosby O, Selmer KK
  Title
CHD2 mutations in Lennox-Gastaut syndrome.
  Journal
Epilepsy Behav 33:18-21 (2014)
DOI:10.1016/j.yebeh.2014.02.005
Reference
  Authors
Shoichet SA, Duprez L, Hagens O, Waetzig V, Menzel C, Herdegen T, Schweiger S, Dan B, Vamos E, Ropers HH, Kalscheuer VM
  Title
Truncation of the CNS-expressed JNK3 in a patient with a severe developmental epileptic encephalopathy.
  Journal
Hum Genet 118:559-67 (2006)
DOI:10.1007/s00439-005-0084-y
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