Prevalence
~15% of children with non-febrile seizures aged 1 to 15 years.

Incidence
10 to 20/100,000 children aged 0 to 15 years.

Age at onset
1 to 14 years, 3/4 start between 7 and 10 years; peak at 8 to 9 years.

Sex
1.5 male preponderance.

Neurological and mental state
Normal.

Genetics
Linkage to chromosome 15q14 in one study. Siblings or parents with the same seizures are rare. Febrile seizures are common (10% to 20%).

Clinical manifestations
Infrequent focal seizures of unilateral facial sensory-motor symptoms, oro-pharyngo-laryngeal manifestations, speech arrest, and hypersalivation.

Hemifacial seizures (1/3 of patients) consist of clonic contractions of the lower lip, sometimes spreading to the arm. Inability to speak and hypersalivation are common concurrent symptoms.

Oro-pharyngo-laryngeal manifestations are unilateral numbness and dysesthesia inside the mouth, cheek, teeth, and tongue alone or usually with motor phenomena producing strange sounds such as death rattle, gargling, guttural sounds, and their combinations.

Consciousness is retained in 58%.

Duration is ~1 to 2 min but lasts longer if seizures progress to convulsions (1/3).

Reversible linguistic and learning abnormalities, usually mild, may be common.

Timing
75% in sleep.

Inter-ictal EEG
Normal background with centrotemporal spikes (CTS), which are abundant, usually in clusters, and markedly accentuated during sleep. In 10% to 20%, these are also evoked by somatosensory stimuli. Rarely, CTS occur only during sleep, the spikes may be very small or the EEG may be normal.

CTS occur in 2% to 3% of normal school-age children and in a variety of organic brain diseases. They are age-dependent and disappear before age 16.

Frequency, location, and persistence of CTS do not determine clinical manifestations, severity, and frequency of seizures or prognosis.

Ictal EEG
Slow waves and spikes, with onset from the Rolandic regions.

Prognosis
Excellent; <2% develop epilepsy. Seizures are infrequent (10% to 20% singular) and remit <16 years of age. 10% to 20% may have frequent seizures.

Less than 1% progress to severe syndromes of linguistic, behavioral, and neuropsychological deficits.

Differential diagnosis
Other epilepsies.

Management options*
Continuous AED therapy may not be needed. Most clinicians use carbamazepine for recurrent seizures though this AED may exceptionally cause marked aggravations. Clobazam#, levetiracetam and sulthiame# are other possible alternatives.

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

See also: http://professionals.epilepsy.com/page/syndromes_benign.html

This page 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