International Classification of Epilepsies and Epileptic Syndromes:

1.1 Idiopathic with age related onsetl Benign childhood epilepsy with centrotemporal spikesl Childhood epilepsy with occipital paroxysms

1.2 Symptomaticl Comprises syndromes of great individual variability, mainly based on:l Anatomical localisationl Clinical featuresl Seizure typesl Etiological factors

(Epileptic syndromes of unknown etiology are classified as cryptogenic)

2.1 Idiopathic, with age related onset, listed in order of agel Benign neonatal familial convulsionsl Benign neonatal convulsionsl Benign myoclonic epilepsy in infancy l Childhood absence epilepsy (pyknolepsy)l Juvenile myoclonic epilepsyl Epilepsy with tonic-clonic seizures on awakening

Other generalised idiopathic epilepsies, if they do not belong to one of theabove syndromes can still be classified as generalised idiopathic epilepsies

2.2 idiopathic and/or symptomatic, in order of age of appearancel Wests syndrome (infantile spasms)l Lennox-Gastaut syndromel Epilepsy with myoclonic-astatic seizuresl Epilepsy with myoclonic absences

2.3 Symptomatic2.3.1 Nonspecific etiology

l Early myclonic encephalopathy

2.3.2 Specific syndromesl Epileptic seizures may complicate many disease statesl Included under this heading are those diseases in which seizures are a presenting or predominant feature

3.1 With both generalised and focal seizuresl Neonatal seizuresl Severe myoclonic epilepsy in infancyl Epilepsy with continuous spike waves during slow wave sleepl Acquired epileptic aphasia (Landau-Kleffner syndrome)

3.2 Without unequivocal generalised or focal featuresl This heading covers all cases where clinical and EEG findings do not

permit classification as clearly generalised or localisation-related, such asin many cases of sleep tonic-clonic seizures

4.1 Situation-related seizuresl Febrile convulsionsl Seizures related to other identifiable situations such as

m Stressm Hormonal changesm Drugsm Alcoholm Sleep deprivation

4.2 Isolated, apparently unprovoked epileptic events4.3 Epilepsies characterised by specific modes of seizure precipitation4.4 Chronic progressive epilepsia partialis continua of childhood

1. Localisation-Related (Focal, Local, Partial)

2. Generalised Epilepsies and Syndromes

3. Epilepsies and Syndromes Undeterminedas to

Whether They Are Focal or Generalised

4. Special Syndromes

From: Epilepsia 1989; 30:389-399

Newbornsl Benign neonatal convulsionsl Familial benign neonatal convulsionsl Early myoclonic encephalopathyl Severe idiopathic status epilepticusl Early infantile epileptic encephalopathy with suppression-burst

Infantsl Febrile convulsionsl Wests syndrome: infantile spasmsl Benign myoclonic epilepsy in infantsl Severe myoclonic epilepsy in infantsl Myoclonic epilepsy (myoclonic status) in nonprogressive encephalopathiesl Epileptic seizures caused by inborn errors of metabolisml Myoclonic-astatic epilepsy of early childhoodl Lennox-Gastaut syndrome

Childrenl Childhood absence epilepsy (pyknolepsy)l Epilepsy with myoclonic absencesl Epilepsy with generalised convulsive seizuresl Benign partial epilepsiesl Benign epilepsy with centrotemporal (rolandic) spikesl Benign psychomotor epilepsyl Benign epilepsy with occipital spike-waves (BEOSW)l Other benign partial epilepsiesl Benign partial epilepsy with extreme somatosensory-evoked potentialsl Landau-Kleffner syndromel Epilepsy with continuous spikes and waves during sleepl Epilepsy with photosensitivityl Eyelid myoclonia absencesl Self-induced epilepsy

Older Children and Adolescents (Juveniles)l Juvenile absence epilepsyl Juvenile myoclonic epilepsy (JME)l Epilepsy with tonic-clonic awakeningl Benign partial seizures of adolescencel Kojewnikoffs syndromel Progressive myoclonus epilepsies

m Juvenile Gauchersm Juvenile neuronal ceroid lipofuscinosis (NCL)m Laforas body disease

l Unverricht-Lundborg disease (Finnish or Baltic myoclonus epilepsy)m Cherry-red spot myoclonus (neuraminidase deficiency)m Dyssynergia cerebellaris myoclonia (Ramsay Hunt syndrome)m Mitochondrial encephalopathy

l Seizure type(s)l EEG (ictal and interictal)l Etiologyl MRI scanl Response to AEDsl Inheritancel Natural history

Paediatric Epilepsy Syndromes by Age of Onset

Factors to Identify the Epileptic Syndrome