Table 4.
Electroclinical syndromes arranged by age at onseta |
Neonatal period |
Benign familial neonatal epilepsy (BFNE) |
Early myoclonic encephalopathy (EME) |
Ohtahara syndrome |
Infancy |
Epilepsy of infancy with migrating focal seizures |
West syndrome |
Myoclonic epilepsy in infancy (MEI) |
Benign infantile epilepsy |
Benign familial infantile epilepsy |
Dravet syndrome |
Myoclonic encephalopathy in nonprogressive disorders |
Childhood |
Febrile seizures plus (FS+) (can start in infancy) |
Panayiotopoulos syndrome |
Epilepsy with myoclonic atonic (previously astatic) seizures |
Benign epilepsy with centrotemporal spikes (BECTS) |
Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) |
Late onset childhood occipital epilepsy (Gastaut type) |
Epilepsy with myoclonic absences |
Lennox-Gastaut syndrome |
Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)b |
Landau-Kleffner syndrome (LKS) |
Childhood absence epilepsy (CAE) |
Adolescence-Adult |
Juvenile absence epilepsy (JAE) |
Juvenile myoclonic epilepsy (JME) |
Epilepsy with generalized tonic-clonic seizures alone |
Progressive myoclonic epilepsies (PME) |
Autosomal dominant epilepsy with auditory features (ADEAF) |
Other familial temporal lobe epilepsies |
Less specific age relationship |
Familial focal epilepsy with variable foci (childhood to adult) |
Reflex epilepsies |
Distinctive constellations |
Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE with HS) |
Rasmussen syndrome |
Gelastic seizures with hypothalamic hamartoma |
Hemiconvulsion-hemiplegia-epilepsy |
Epilepsies that do not fit into any of these diagnostic categories can be distinguished first on the basis of the presence or absence of a known structural or metabolic condition (presumed cause) and then on the basis of the primary mode of seizure onset (generalized vs. focal) |
Epilepsies attributed to and organized by structural-metabolic causes |
Malformations of cortical development (hemimegalencephaly, heterotopies, etc.) |
Neurocutaneous syndromes (tuberous sclerosis complex, Sturge-Weber, etc.) |
Tumor |
Infection |
Trauma |
Angioma |
Perinatal insults |
Stroke |
Etc. |
Epilepsies of unknown cause |
Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy |
Benign neonatal seizures (BNS) |
Febrile seizures (FS) |
The arrangement of electroclinical syndromes does not reflect etiology,
Sometimes referred to as Electrical Status Epilepticus during Slow Sleep (ESES),
Reference: Berg AT et al. Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010; 51(Supll 4):676–685.