Lennox–Gastaut syndrome
| Lennox–Gastaut syndrome | |
|---|---|
| Generalized 2.5 Hz spike and wave discharges in a child with childhood absence epilepsy | |
| Specialty | Neurology |
Lennox–Gastaut syndrome (LGS) is a complex, rare, and severe childhood-onset epilepsy syndrome. It is characterized by multiple and concurrent seizure types, including tonic seizure, cognitive dysfunction, and slow spike waves on electroencephalogram (EEG), which are very abnormal.[1] Typically, it presents in children aged 3–5 years and most of the time persists into adulthood with slight changes in the electroclinical phenotype.[2][3] It has been associated with perinatal injuries, congenital infections, brain malformations, brain tumors, genetic disorders such as tuberous sclerosis and numerous gene mutations. Sometimes LGS is observed after infantile epileptic spasm syndrome (formerly called West syndrome). The prognosis for LGS is marked by a 5% mortality in childhood and persistent seizures into adulthood (at least 90% of adults with LGS still have seizures).[4]
LGS was named for neurologists William G. Lennox (Boston, US) and Henri Gastaut (Marseille, France),[5] who independently described the condition. The international LGS Awareness Day is on November 1.[6]
- ^ Specchio, Nicola; Wirrell, Elaine C.; Scheffer, Ingrid E.; Nabbout, Rima; Riney, Kate; Samia, Pauline; Guerreiro, Marilisa; Gwer, Sam; Zuberi, Sameer M.; Wilmshurst, Jo M.; Yozawitz, Elissa; Pressler, Ronit; Hirsch, Edouard; Wiebe, Samuel; Cross, Helen J. (June 2022). "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". Epilepsia. 63 (6): 1398–1442. doi:10.1111/epi.17241. ISSN 0013-9580. PMID 35503717. S2CID 248503776.
- ^ Markand, Omkar N. (2003-12-01). "Lennox–Gastaut syndrome (childhood epileptic encephalopathy)". Journal of Clinical Neurophysiology. 20 (6): 426–441. doi:10.1097/00004691-200311000-00005. ISSN 0736-0258. PMID 14734932. S2CID 46515787.
- ^ Archer, John S.; Warren, Aaron E. L.; Jackson, Graeme D.; Abbott, David F. (2014-01-01). "Conceptualizing Lennox–Gastaut syndrome as a secondary network epilepsy". Frontiers in Neurology. 5: 225. doi:10.3389/fneur.2014.00225. PMC 4214194. PMID 25400619.
- ^ Asadi-Pooya, Ali A. (March 2018). "Lennox-Gastaut syndrome: a comprehensive review". Neurological Sciences. 39 (3): 403–414. doi:10.1007/s10072-017-3188-y. ISSN 1590-3478. PMID 29124439. S2CID 4243468.
- ^ Dravet, C., & Roger, J. (1996). Henri Gastaut, 1915–1995. Epilepsia, 37(4), 410–415. https://doi.org/10.1111/j.1528-1157.1996.tb00580.x
- ^ "LGS Foundation | Lennox–Gastaut Syndrome".