Page 117 - ILAE_Lectures_2015

Table 1. Classification of generalised epilepsies and epileptic syndromes2.
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2.1 Idiopathic generalised age-related
2.1.1 Benign neonatal familial convulsions
2.1.2 Benign neonatal convulsions
2.1.3 Benign myoclonic epilepsy in infancy
2.1.4 Childhood absence epilepsy (pyknolepsy)
2.1.5 Juvenile absence epilepsy
2.1.6 Juvenile myoclonic epilepsy (impulsive petit mal)
2.1.7 Epilepsy with grand mal (generalised tonic-clonic) seizures on awakening
2.1.8 Other generalised idiopathic epilepsies not defined above
2.1.9 Epilepsies with seizures precipitated by specific modes of activation

2.2 Cryptogenic or symptomatic generalised
2.2.1 West syndrome (infantile spasms, Blitz-Nick-Salaam Krampfe)
2.2.2 Lennox-Gastaut syndrome
2.2.3 Epilepsy with myoclonic-astatic seizures
2.2.4 Epilepsy with myoclonic absences

2.3 Symptomatic generalised
2.3.1 Non-specific aetiology
2.3.1.1 Early myoclonic encephalopathy
2.3.1.2 Early infantile myoclonic encephalopathy with suppression burst
2.2.1.3 Other symptomatic generalised epilepsies not defined above
2.3.2 Specific syndromes
2.3.2.1 Specific diseases in which seizures are the presenting feature
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The significance of specifying the type of ‘epilepsy’

The significance and the challenges of the syndromic classification of epilepsies is
exemplified by three common epileptic syndromes: benign childhood seizure susceptibility
syndromes, juvenile myoclonic epilepsy and syndromes of temporal lobe epilepsy that
comprise more than 40% of all epilepsies. They are entirely different in presentation, cause
and genetics, investigative procedures, short and long-term treatment strategies and
prognosis.

Benign childhood seizure susceptibility syndromes
Benign childhood seizure susceptibility syndromes (BCSSS) are detailed in Chapter 9. They
comprise one-quarter of all childhood epilepsies. Like febrile convulsions, BCSSS are age-
related, show genetic predisposition, may be manifested by a single seizure, remit within a
few years of onset, and may or may not require a short course of antiepileptic medication.
The risk of recurrent seizures in adult life (12%) is less than in febrile convulsions (4%).
Recognition of the characteristic clinical and EEG features of BCSSS enable parents to be
reassured of the invariably benign prognosis with spontaneous resolution of the disorder by
the mid-teens6,19.

Juvenile myoclonic epilepsy
Juvenile myoclonic epilepsy (JME) is an idiopathic generalised epileptic syndrome with
distinctive clinical and EEG features12,20,21. Prevalence is 810% among adult patients with
seizures12,20,21. It is characterised by myoclonic jerks on awakening, generalised tonic-clonic