Benign childhood seizure susceptibility syndromes

Benign childhood focal seizures and related idiopathic epileptic syndromes affect 25% of children with non-febrile seizures and constitute a significant part of the everyday practice of paediatricians, neurologists and electroencephalographers. They comprise three identifiable electroclinical syndromes recognised by the International League against Epilepsy (ILAE)1: rolandic epilepsy which is well known; Panayiotopoulos syndrome (PS), a common autonomic epilepsy, which is currently more readily diagnosed; and the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) including the idiopathic photosensitive occipital lobe epilepsy, a less common form with uncertain prognosis. There are also reports of children with benign focal seizures of predominantly affective symptoms, and claims have been made for other clinical phenotypes associated with specific inter-ictal EEG foci, such as frontal, midline or parietal, with or without giant somatosensory evoked spikes (GSES). Neurological and mental states and brain imaging are normal, though because of their high prevalence any type of benign childhood focal seizures may incidentally occur in children with neurocognitive deficits or abnormal brain scans. The most useful diagnostic test is the EEG. In clinical practice, the combination of a normal child with infrequent seizures and an EEG showing disproportionately severe spike activity is highly suggestive of these benign childhood syndromes2.