Prevalence
13% of children with non-febrile seizures aged 3 to 6 years, 6% in the age group 1 to 15 years.

Age at onset
3 to 6 years in 74% of cases; range 1 to 14 years.

Sex
Males = females.

Neurological and mental state
Normal.

Genetic and other factors
Febrile seizures in ~17%. Occurrence in other siblings is exceptional. High incidence of abnormal birth deliveries.

Clinical manifestations
The seizures mainly manifest with autonomic symptoms. Emesis often culminating to vomiting is the most common (70% to 80%) but is not always present or apparent. Others include pallor, incontinence of urine and feces, mydriasis or miosis, cardio-respiratory irregularities, hypersalivation, thermoregulatory changes, and cephalic sensations. Ictal syncope (unresponsive and flaccid) is common before or without convulsions.

Behavioral ictal features are irritability or quietness, patient looks or feels unwell.

Consciousness is usually intact at onset but severely disturbed in the seizure progression.

Other ictal features include eye deviation (60% to 80%); half progress to hemiconvulsions or generalized convulsions. Visual hallucinations are rare (10%) and do not appear at the onset.

Duration is long, typically 5 to 10 min; nearly half have autonomic status epilepticus (>30 min).

Timing
2/3 of the seizures occur in sleep.

Inter-ictal EEG
Multifocal spikes are the more common; occipital spikes +/- extra-occipital spikes predominate, extra-occipital spikes (1/3) or brief generalized discharges may occur, 1/10 have normal EEG.

Ictal EEG
Focal slow waves intermixed with spikes. May start posteriorly or anteriorly.

Prognosis
Excellent, remission within 1 to 2 years, 1/3 of patients have a single seizure, only ~10% have >10 seizures; 1/5 (21%) may develop other seizure types, mainly Rolandic (13%), which are usually infrequent and remit before the age of 16 years. Risk for later epilepsy is the same as in normal population. However, exceptionally autonomic seizures may be life-threatening causing cardio-respiratory arrest.

Differential diagnosis
Encephalitis, atypical migraine, syncope, gastroenteritis, unspecified epilepsy, febrile convulsions, sleep disorder, motion sickness.

Management options*
Continuous AED therapy is not recommended for children with a single seizure or brief seizures. For recurrent seizures, most clinicians use carbamazepine. Clobazam#, levetiracetam and sulthiame# are other possible alternatives. Lengthy seizures are a medical emergency; rectal diazepam is prescribed for home administration.

*Expert opinion, please check FDA-approved indications and prescribing information
#Not approved by the FDA

This section was adapted from:

The educational kit on epilepsies: The epileptic syndromes By C. P. Panayiotopoulos Originally published by MEDICINAE, 21 Cave Street, Oxford OX4 1BA

First published 2006 and reprinted in 2007. The Educational Kit on Epilepsies was produced through an unrestricted educational grant from UCB Pharma SA.

UCB Pharma SA assumes no responsibility of the views expressed and recommended treatments in these volumes.

Authored by: C. P. Panayiotopoulos MD PhD FRCP on 1/2005
Reviewed by: Steven C. Schachter MD on 6/2008
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