H01813 | |
H number | H01813 |
Name | Lennox-Gastaut syndrome |
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 |
Network | - |
Gene | MAPK10 [HSA:5602] [KO:K04440] |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Fenfluramine hydrochloride [DR:D04148] Clonazepam [DR:D00280] Rufinamide [DR:D05775] Lamotrigine [DR:D00354] Topiramate [DR:D00537] Cannabidiol [DR:D10915] Clobazam [DR:D01253] |
Comment | - |
Other DBs | ICD-11: 8A62.1 ICD-10: G40.4 MeSH: D065768 OMIM: 606369 |
Reference | PMID:25284032 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 PMID:19589458 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 PMID:24614520 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 PMID:16249883 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 |