Table 4

A summary of the clinical features of the neonatal epilepsy syndromes

Neonatal epilepsy syndromeAxis 1 (ictal phemenology—what you see)Axis 2 (seizure types)EEG findingsAxis 4 (aetiology)TreatmentAxis 5 (prognosis/disability)
Benign neonatal seizuresRhythmical jerking of the arms and legs. Sometimes facial involvement on days 4–6 of life8 9Focal, multifocal, hemiconvulsive clonic seizuresNon-specific including:36
▸ Theta pointu alternants
▸ Multifocal abnormalities
▸ Discontinuous EEG pattern
▸ Rhythmical spike/slow waves in rolandic regions
UnknownIntravenous benzodiazepine, phenytoin acutely
None in long term
Seizures abate8 9
Benign familial neonatal seizuresShort, frequent stiffenings with apnoea or crying. May have desaturation or bradycardia10 37Tonic seizures with autonomic features. Less frequently clonic seizures alongside tonic seizuresNon-specific including:10 36
▸ Normal
▸ Discontinuous
▸ Focal or multifocal abnormalities
▸ Theta pointu alternants
▸ Flattening of EEG trace initially
▸ Then bilateral, asymmetric spike and sharp waves
Autosomal-dominant channelopathy (strong family history).
OMIM: 608217
OMIM: 121200
OMIM: 121201
Rare autosomal-recessive OMIM: 269720
Intravenous benzodiazepine, phenytoin acutely
May require long-term treatment
Seizures abate by 6 months usually. 14% have longer-term seizures.
Developmental outcome good36 37
Early infantile epileptic encephalopathy (Ohtahara syndrome)Sick baby. Seizures before or after birth (average day 10). Tonic stiffening in clusters with desaturation, sometimes due to diaphragmatic splinting11Focal or generalised tonic seizures with autonomic features.
Less common—focal clonic seizures
Burst suppression in wake and sleep states11Structural brain abnormalities most common.11–13
Genetic abnormalities14
None effective
Consider vitamins, vigabatrin, ketogenic diet15–17
Epilepsy surgery19
Dire if no surgically resectable lesion. Half die within weeks.
Survivors invariably have neurological/learning difficulties and epilepsy including West and Lennox Gastaut syndromes11
Early myoclonic epilepsy (EME)Sick baby.
Rapid jerks of limbs, face and trunk. May come in clusters but not rhythmical11
Myoclonic seizures
Also, focal clonic, autonomic and tonic seizures
Burst suppression more apparent in sleep than wake state11Genetic causes, metabolic aetiologies most common11None effective.
Consider vitamins and ketogenic diet20
Dire. More than half die within weeks or months.
Survivors invariably have neurological/learning difficulties and epilepsy11
Migrating partial seizures of infancyUsually presents in infancy, rarely in neonatal period.
Frequent tonic deviation of eyes and head to the side with eye lid jerking, and stiffening and clonic jerking of limbs. May have autonomic features.
Seizures move around the body22 36
Focal tonic, focal clonic and autonomic seizures.
Migrating seizures
▸ Slow activity alternating sides
▸ Multifocal spikes
▸ Rhythmical theta and alpha discharges multifocally and migrating to different brain areas
▸ Several seizures occurring independently in different regions of brain36
Genetic aetiology most likely22Consider vitamins
For apnoea—try acetazolamide
▸ Stiripentol and clobazam
▸ Potassium bromide
▸ Levetiracetam
▸ Rufinamide
▸ Quinidine
▸ Ketogenic diet23–28
Dire. Long-term neurological, developmental difficulties and epilepsy
Death usually within a few years of life25