Page 100 - ILAE_Lectures_2015

Benign childhood seizures with affective symptoms
Benign childhood epilepsy with affective symptoms, reported in less than 40 patients, is a
clinical phenotype of BCSSS with features common in both PS (behavioural and autonomic
symptoms) and rolandic epilepsy (speech arrest and hypersalivation)156,157. Onset is between 2–
9 years of age and both sexes are equally affected.

Seizures manifest with terror and screaming, autonomic disturbances (pallor, sweating,
abdominal pain, hypersalivation), chewing and other automatisms, speech arrest and mild
impairment of consciousness. These are usually brief for 1–2 minutes, frequently occurring
several times a day in wakefulness or sleep. One-fifth of patients have febrile seizures and some
may also have infrequent rolandic seizures. Generalised seizures do not occur.

The inter-ictal EEG shows high-amplitude frontotemporal and parietotemporal spikes that are
exaggerated by sleep. Ictal EEG discharges are mainly localised in the frontotemporal,
centrotemporal or parietal regions and are stereotypical for each patient.

The response to treatment is excellent and remission occurs within 1–2 years from onset.
Behavioural problems may be prominent during the active stage of the disease, but subside later
with seizure remittance.

Benign childhood epilepsy with parietal spikes and frequent extreme somatosensory-evoked
spikes
Benign childhood epilepsy with parietal spikes and frequent GSES46,47,158 has been proposed as
another phenotype of BCSSS. The defining features are EEG spikes in the parietal regions,
which are often elicited by tactile stimulation. However, GSES are not specific for any
syndrome because they also occur in 10–20% of children with rolandic seizures47, in a few
patients with PS2,4 and in children with no seizures159.

Versive seizures of the head and body, often without impairment of consciousness, are mainly
diurnal and infrequent. Frequent seizures and focal status epilepticus are exceptional.

Remission usually occurs within one year from seizure onset, but EEG abnormalities may
persist for longer.

Benign childhood focal seizures associated with frontal or midline spikes
Benign childhood focal seizures associated with frontal2,160,161 or midline spikes2,162 have been
described and long follow-up reports have confirmed a benign course, although no systematic
studies have been published. However, EEG spike foci specificity is questionable, as EEG spike
foci of various locations (including frontal and midline) are also seen in rolandic epilepsy and
more commonly in PS, and midline spikes are more common in children than in adults163,164.

Recently ‘benign infantile focal epilepsy with midline spikes during sleep’ has been described
as a new syndrome of BCSSS165,166. Age at onset is in the first three years of life and both sexes
are equally affected. Seizures consist mainly of staring, motion arrest, cyanosis, loss of
consciousness and stiffening of the arms. Clonic convulsions and automatisms are rare.
Seizures are brief from 1–5 minutes, mainly diurnal and are generally infrequent from 1–3 per
year. There is a strong family history of undefined types of epileptic seizures with benign
epilepsies prevailing.

Inter-ictal EEG abnormalities are seen only in non-REM sleep and consist of small, mostly
singular, midline spikes. The prognosis is excellent, with remission of seizures, normal
development and normalisation of the EEG before the age of four years.