idiopathic susceptibility to early onset benign childhood seizures with electroencephalograhic occipital or extra occipital spikes, and manifests mainly with autonomic seizures. It is a benign childhood epilepsy
affects about 13% of children of 3-6 years old with one or more non-febrile seizures (peak age 4-5 years)
affects about 6% of the age group 1-15 years
more common in girls (2)
presenting in early childhood (mean 4-7 years) with rare seizures that are prolonged
characterised by autonomic features including vomiting, pallor and sweating followed by tonic eye deviation, impairment of consciousness with possible evolution into a secondarily generalised seizure
prognosis is excellent and treatment often unnecessary (3)
seizures can be prolonged, may mimic non-epileptic disorders, and may vary in severity from trivial to apparently life threatening
autonomic seizures are the hallmark of the Panayiotopoulos syndrome
autonomic symptoms and signs (mainly vomiting) occur from the onset in 80% of seizures, with half of them lasting for more than 30 minutes to hours, thus amounting to autonomic status epilepticus
about two thirds of the seizures occur during nocturnal sleep or brief daytime naps. "In a typical daytime seizure the child looks pale, complains, "I want to be sick," and vomits. If in sleep, the child wakes up with similar complaints or is found vomiting, confused, or unresponsive (1) ".Vomiting occurs in about 75% of seizures
other autonomic manifestations may occur either concurrently with vomiting or later in the course of the seizure, and include mydriasis, pallor, cardiorespiratory, gastrointestinal and thermoregulatory alterations, incontinence, and hypersalivation. In 20% or more of the seizures the child becomes pale, unresponsive and flaccid either before convulsing or in isolation
of nonautonomic manifestations, lateral eye deviation appears the most common (2)
seizures during sleep seem more common than those in wakefulness (2)
it is characteristic, even after the most severe seizures and status, for the the child to be normal after a few hours of sleep
EEG - generally shows multifocal spikes at various locations - normal in one third of patients
clinical course - benign - remission generally occurs within two years from onset
33% of these children have a single seizure, and only 5-10% have more than 10 seizures that may be very frequent sometimes but the outcome is still favourable
Lengthy seizures do not appear to result in residual deficits or have adverse prognostic significance
about 20% of children with this syndrome may develop other types of infrequent, usually rolandic seizures, but these also remit before the age of 16 years
management
first-line treatment in children and young people with benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) (3)
discuss with the child or young person, and their family and/or carers, whether AED treatment for benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) is indicated
carbamazepine or lamotrigine should be offered as first-line treatment to children and young people with benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type)
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