One example of how syndromes can be organized:Arranged by typical age at onset*Electroclinical syndromesDistinctive constellations/surgical syndromesNeonatal periodBenign neonatal seizuresˆBenign familial neonatal epilepsy(BFNE)Ohtahara syndromeEarly myoclonicencephalopathy(EME)Distinctive constellations/surgical syndromesMesial temporal lobe epilepsy with hippocampalsclerosis (MTLE with HS)Rasmussen syndromeGelastic seizures with hypothalamic hamartomaHemiconvulsion-hemiplegia-epilepsyNonsyndromic epilepsies**Epilepsies attributed to and organizedby structural-metabolic causesMalformations of cortical development(hemimegalencephaly, heterotopias, etc.)Neurocutaneous syndromes (tuberous sclerosiscomplex, Sturge-Weber, etc.)Tumor, infection, trauma, angioma, antenataland perinatal insults, stroke, etc.Epilepsies of unknown causeInfancyFebrile seizuresˆ, Febrile seizures plus (FS+)Benign infantile epilepsyBenign familial infantile epilepsy(BFIE)West syndromeDravet syndromeMyoclonic epilepsy in infancy (MEI)Myoclonic encephalopathy innonprogressivedisordersEpilepsy of infancy with migratingfocal seizuresAdolescence AdultJuvenile absence epilepsy (JAE)Juvenile myoclonicepilepsy (JME)Epilepsy with generalized tonic-clonic seizures aloneAutosomal dominantepilepsy with auditoryfeatures (ADEAF)Other familial temporallobe epilepsiesVariable age at onsetFamilial focal epilepsywith variable foci(childhood to adult)Progressive myoclonus epilepsies(PME)Reflex epilepsiesChildhoodFebrile seizuresˆ, Febrile seizures plus (FS+)Early onset childhood occipital epilepsy (Panayiotopoulossyndrome)Epilepsy with myoclonic atonic(previously astatic) seizuresChildhood absence epilepsy (CAE)Benign epilepsy with centrotemporal spikes (BECTS)Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE)Late onset childhood occipitalepilepsy (Gastaut type)Epilepsy with myoclonic absencesLennox-Gastaut syndrome (LGS)Epileptic encephalopathy withcontinuous spike-and-wave during sleep (CSWS)+Landau-Kleffner syndrome (LKS)
Figure 15-2. ILAE proposal for revised terminology for organization of seizures and epilepsies 2010 electroclinical syndromes and other epilepsies grouped by specificity of diagnosis. (From http://www.ilae.org/Visitors/Centre/ctf/documents/ILAEHandoutV10_000.pdf.) 6050403020100Complex partialSimple partialPartial, unclassifiedGeneralized tonic-clonicAbsenceMyoclonicGeneralized, otherUnknown, multiple0–1415–3435–64YearsType of seizurePercent of incident cases>65
Figure 15-3. The distribution of the main types of epilepsy by age. The overrepresentation of absence and myoclonic seizures in childhood and of complex partial seizures in older individuals is evident. Complex partial=focal with dyscognitive features; simple partial=focal without dyscognitive features. (Adapted from Hauser and Annegers and the texts of Engel and of Pedley.)