Table 1.5

Syndromic Classification of the ILAE 57

1. Localization-related (focal, local, partial) epilepsies and syndromes
1.1 Idiopathic (with age-related onset)
 At present, the following syndromes are established, but more may be identified in the future:
  • Benign childhood epilepsy with centrotemporal spike
  • Childhood epilepsy with occipital paroxysms
  • Primary reading epilepsy
1.2 Symptomatic
  • Chronic progressive epilepsia partialis continua of childhood (Kozhevnikov syndrome)
  • Syndromes characterized by seizures with specific modes of precipitation
  • Temporal lobe epilepsies
  • Frontal lobe epilepsies
  • Parietal lobe epilepsies
  • Occipital lobe epilepsies
1.3 Cryptogenic
 Cryptogenic epilepsies are presumed to be symptomatic and the aetiology is unknown. This category thus differs from the previous on by the lack of etiologic evidence (See definitions).
2. Generalized epilepsies and syndromes
2.1 Idiopathic (with age-related onset – listed in order of age)
  • Benign neonatal familial convulsions
  • Benign neonatal convulsions
  • Benign myoclonic epilepsy in infancy
  • Childhood absence epilepsy (pyknolepsy)
  • Juvenile absence epilepsy
  • Juvenile myoclonic epilepsy (impulsive petit mal)
  • Epilepsy with grand mal (GTCS) seizures on awakening
  • Other generalized idiopathic epilepsies not defined above
  • Epilepsies with seizures precipitated by specific modes of activation
2.2 Cryptogenic or symptomatic (in order of age)
  • West syndrom (infantile spasms, Blitz-Nick-Salaam Krampfe)
  • Lennox-Gastaut syndrome
  • Epilepsy with myoclonic-astatic seizures
  • Epilepsy with myoclonic absences
2.3 Symptomatic 2.3.1 Non-specific aetiology
  • Early myoclonic encephalopathy
  • Early infantile epileptic encephalopathy with suppression burst
  • Other symptomatic generalized epilepsies not defined above
 2.3.2 Specific syndromes
  • Epileptic seizures may complicate many disease states. Under this heading are included diseases in which seizures are a presenting or predominant
3. Epilepsies and syndromes undetermined whether focal or generalized
3.1 With both generalized and focal seizures
  • Neonatal seizures
  • Severe myoclonic epilepsy in infancy
  • Epilepsy with continuous spike-waves during slow wave sleep
  • Acquired epileptic aphasia (Landau-Kleffner syndrome)
  • Other undetermined epilepsies not defined above
3.2 Without unequivocal generalized or focal features. All cases with generalized tonic-clonic seizures in which clinical and EEG findings do not permit classification as clearly generalized or localization related, e.g., many cases of sleep-grand mal (GTCS) are considered not to have unequivocal generalized or focal features
4. Special syndromes
4.1 Situation-related seizures (Gelegenheitsanfälle)
  • Febrile convulsions
  • Isolated seizures or isolated status epilepticus
  • Seizures occurring only when there is an acute metabolic or toxic event due to factors such as alcohol, drugs, eclampsia, nonketotic hyperglycaemia

From: The Commission on Classification and Terminology of the International League Against Epilepsy.

Proposal for revised clinical and electroencephalographic classification of epileptic seizures. With the permission of the Editor of Epilepsia.55

From: Chapter 1, Clinical Aspects of the Diagnosis of Epileptic Seizures and Epileptic Syndromes

Cover of The Epilepsies
The Epilepsies: Seizures, Syndromes and Management.
Panayiotopoulos CP.
Oxfordshire (UK): Bladon Medical Publishing; 2005.
Copyright © 2005, Bladon Medical Publishing, an imprint of Springer Science+Business Media.

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