Neonatal epilepsy syndromes

see also neonatal seizures

Despite the high prevalence of neonatal seizures, epileptic syndromes in neonates are rare. The following four syndromes have been recognised by 1989 ILAE classification.

• benign neonatal familial convulsions
• benign neonatal convulsions
• early myoclonic encephalopathy
• early infantile epileptic encephalopathy with suppression burst

These four neonatal syndromes were retained (with some modifications in their names) in the ILAE Task Force diagnostic scheme. In the ILAE report of 2006, the recognised epileptic symdromes in the neonatal period are:

• benign familial neonatal seizures: this may be a disease and not a syndrome (level 3 recommendation of confidence)
• early myoclonic encephalopathy: although this may be different from Ohtahara syndrome, the clinical distinction can be difficult (level 3 recommendation of confidence).
• Ohtahara syndrome: (level 3 recommendation of confidence).
• Benign neonatal seizures (non-familial) are categorised among ‘conditions with epileptic seizures that do not require a diagnosis of epilepsy’.

Clarifications on classification

• the 1989 ILAE classification considers:‘benign familial neonatal convulsions’ and ‘benign neonatal convulsions (non-familial)’ as ‘idio pathic generalised epilepsies (age related)’
• the new ILAE reports abandon the name convulsions, using instead seizures
• ‘early myoclonic encephalopathy’ and ‘Ohtahara syndrome’ as ‘generalised symptomatic epilepsies of non-specific aetiology (age related)’
• the new ILAE reports classify them as ‘epileptic encephalopathies (in which the epileptiform abnormali ties may contribute to progressive dysfunction)’