A summary of the clinical features of the neonatal epilepsy syndromes
| Neonatal epilepsy syndrome | Axis 1 (ictal phemenology—what you see) | Axis 2 (seizure types) | EEG findings | Axis 4 (aetiology) | Treatment | Axis 5 (prognosis/disability) |
|---|---|---|---|---|---|---|
| Benign neonatal seizures | Rhythmical jerking of the arms and legs. Sometimes facial involvement on days 4–6 of life8 9 | Focal, multifocal, hemiconvulsive clonic seizures | Non-specific including:36 Interictal: ▸ Theta pointu alternants ▸ Multifocal abnormalities ▸ Discontinuous EEG pattern Ictal: ▸ Rhythmical spike/slow waves in rolandic regions | Unknown | Intravenous benzodiazepine, phenytoin acutely None in long term | Good. Seizures abate8 9 |
| Benign familial neonatal seizures | Short, frequent stiffenings with apnoea or crying. May have desaturation or bradycardia10 37 | Tonic seizures with autonomic features. Less frequently clonic seizures alongside tonic seizures | Non-specific including:10 36 Interictal: ▸ Normal ▸ Discontinuous ▸ Focal or multifocal abnormalities ▸ Theta pointu alternants Ictal: ▸ 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 | Good. 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 splinting11 | Focal or generalised tonic seizures with autonomic features. Less common—focal clonic seizures | Burst suppression in wake and sleep states11 | Structural 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 state11 | Genetic causes, metabolic aetiologies most common11 | None 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 infancy | Usually 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 | Interictal: ▸ Slow activity alternating sides ▸ Multifocal spikes Ictal: ▸ Rhythmical theta and alpha discharges multifocally and migrating to different brain areas ▸ Several seizures occurring independently in different regions of brain36 | Genetic aetiology most likely22 | Consider vitamins For apnoea—try acetazolamide Others: ▸ 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 |