Several studies have demonstrated that seizure manifestations evolve according to developmental age. Although myelination is largely complete by two years of age, clinical seizure manifestations continue to evolve beyond this age (probably reflecting the caudorostral pattern of brain development), with an adult pattern of semiology unlikely before age 6 years. A high index of suspicion is therefore required when evaluating the clinical description of events for focality. The assessment will have obvious management implications, particularly for the diagnosis of an epilepsy syndrome. Most studies evaluating seizure semiology have been performed in surgical cohorts, where documentation of seizure type is made with video EEG telemetry and confirmed by seizure freedom after removal of the suspect area.

One study examined the localizing and lateralizing value of behavior change in focal seizures in childhood and found it was much more likely at seizure onset for children under age 6 years, occurring in 46% (25/56) compared to only 8/53 in the older group.¹ Behavioral change was far more likely to be of an affective type (agitation, fearful expression, looking for shelter) than an arrest type. Both could represent an aura that could not be verbalized by children in this age group.

In another study of autonomic symptoms (respiratory, gastrointestinal, cutaneous, papillary, urinary) in children age 10 months to 12 years with temporal and extratemporal focal epilepsy, 60/100 patients produced at least one autonomic symptom during their seizures: 43 (70%) of 61 with temporal lobe epilepsy and 17 (44%) of 39 with extratemporal epilepsy. Apnea/bradycardia were more frequent in children <3 years² with temporal lobe onset. Only twelve reported an epigastric aura, the youngest presenting at age 5.3 years reported as abdominal pain or discomfort. It was most frequently associated with temporal lobe onset, and was not lateralizing.

Age dependence has also been demonstrated in ictal automatisms, lateralizing signs and secondary generalization in studies of children with temporal lobe onset seizures.³ However emotional expressions and autonomic signs did not show age dependent manifestation. Emotional expressions (fear, crying, smile, pain, happiness, and laughing) appear more frequently in extratemporal (49%) than temporal (26%) lobe seizures.⁴ Positive emotional expressions were more frequently associated with right sided seizure onset.

Frontal lobe semiology under the age of 7 years is characterized by high seizure frequency (up to 40 per day), approximately half of whom show a tendency to cluster. In a study of 111 seizures in 14 patients, 47% attacks arose from sleep and were of short duration; no correlation was seen between age and duration.⁵ Auras were infrequent. Motor manifestations were most common. All had motor seizures; in fact, only 6 of 11 analyzed attacks displayed no motor signs at all. Besides tonic seizures, clonic components and epileptic spasms were the leading manifestations. Epileptic spasms typically began from age 2 to 16 months, and persisted well beyond infancy. Psychomotor seizures were rare. Behavior change however was seen frequently, with 36% showing some form of vocalization (crying, moaning, and grunting).

A study of children with frontal lobe epilepsy (FLE) and others with seizures arising from the posterior cortex showed little difference in seizure frequency, but revealed a nocturnal predominance in those with FLE.⁶ Visual aura, nystagmus and versive seizures were observed exclusively in the posterior onset group, whereas somatosensory aura and hypermotor seizures appeared only in FLE but less frequently than adults. Tonic seizures were more frequent in FLE. Both groups showed myoclonic seizures, epileptic spasms, psychomotor and atonic seizures, oral and manual automatisms as well as vocalization and eye deviation. Characteristic features of focal onset seizures described in adults with extratemporal epilepsy are frequently missing during childhood, especially infants and school children. In contrast to adults, secondarily generalized seizures are rare.

There are few syndromes with focal seizures as a prominent feature that present at this age. Panayiotopolous syndrome is regarded as a common childhood onset epilepsy involving a prominence of autonomic seizures. Peak age of onset is between 4–5 years; presentation with a single episode of status epilepticus is common. Seizures manifest with a constellation of behavior change, vomiting, color change, and other more typical ictal manifestations. Formerly thought to have an occipital onset, autonomic symptoms are more prominent and therefore it is now more preferably considered an autonomic epilepsy. However although the prognosis for seizure remission in this particular type of epilepsy is extremely good, a low threshold should be maintained for detailed neuroimaging in view of overlap with some of the semiology with that presenting from occipital structural abnormalities (see Chapter 26).

Benign epilepsy with centrotemporal spikes (BECTS) has a peak onset between 5 and 8 years. The seizures are characterized by focal motor involvement of the face and upper limb although secondarily generalized tonic clonic seizures may occur from sleep. This typical pattern of presentation, along with recognized centrotemporal spikes on the waking or sleep EEG facilitates the diagnosis. However the presence of atypical features should prompt the need to obtain brain imaging. The prognosis for seizure remission is extremely good, and discussion often centers on the need for treatment rather than which medication (see Chapter).

Late onset childhood occipital epilepsy of Gastaut type is characterized by focal seizures commonly involving elementary visual hallucinations. The consistency and stereotyped nature of the hallucinations often enables the child to draw them. Ictal blindness is also common and either occurs de novo or follows the hallucinations. Consciousness is not impaired but may be impaired or lost during the later stages of the seizure that often progresses to hemi or bilateral convulsions. Onset is from 3–15 years with a mean of 8 years. Prognosis for seizure control is good with remission within 2–4 years from onset in about 50–60% cases (see Chapter). The differential diagnosis includes migraine in view of the high frequency of postictal headache.