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Hypersalivation, a prominent autonomic manifestation, is often associated with hemifacial
seizures, oro-pharyngo-laryngeal symptoms and speech arrest. The child is actually anarthric,
unable to utter a single intelligible word and attempts to communicate with gestures.

Consciousness and recollection are fully retained in more than half (58%) of rolandic seizures.
In the remainder, consciousness becomes impaired during the ictal progress and in one-third
there is no recollection of ictal events. Progression to hemiconvulsions or generalised tonic–
clonic seizures (GTCS) occurs in around half of children and hemiconvulsions may be followed
by post-ictal Todd’s hemiparesis2. Consciousness and recollection are fully retained in more
than half (58%) of rolandic seizures. In the remainder, consciousness becomes impaired during
the ictal progress and in one-third there is no recollection of ictal events.

Three-quarters of rolandic seizures occur during non-REM (rapid eye movement) sleep, mainly
at sleep onset or just before awakening.

Rolandic seizures are usually brief, lasting for 1–3 minutes. Focal motor, hemiconvulsive and
generalised convulsive status epilepticus are rare at around 5%2,21,22. Opercular status
epilepticus usually occurs in children with atypical evolution23-25 or may be induced by
carbamazepine or lamotrigine26,27. This state lasts for hours to months and consists of ongoing
unilateral or bilateral contractions of the mouth, tongue or eyelids, positive or negative subtle
perioral or other myoclonus, dysarthria, speech arrest, difficulties in swallowing, buccofacial
apraxia and hypersalivation.

Other seizure types
Despite prominent hypersalivation, focal seizures with primarily autonomic manifestations
(autonomic seizures) are not considered part of the core clinical syndrome of rolandic epilepsy.
However, some children may present with independent autonomic seizures or seizures with
mixed rolandic-autonomic manifestations including emesis (see below, relations between
rolandic epilepsy and PS).

Primarily GTCS are considered part of rolandic epilepsy by the ILAE1 and their occurrence
cannot be excluded. However, from the published ictal recordings2,10,28 and the electroclinically
unequivocal focal nature of rolandic epilepsy, it can be inferred that at least the majority of the
GTCS follow rolandic activation, and are therefore secondarily GTCS. Short-lived initial focal
symptoms may pass unnoticed in daytime GTCS and are bound to be missed in nocturnal
GTCS.

Electroencephalography
By definition, centrotemporal spikes (CTS) are the hallmark of benign childhood epilepsy with
CTS. However, although called centrotemporal, these spikes are mainly localised in the C3 and
C4 (high central) or C5 and C6 (low central) supra-sylvian and not temporal electrodes2,29. CTS
are often bilateral and typically activated by drowsiness and slow (non-REM) sleep, but not by
overbreathing. Rarely, children with rolandic epilepsy may have normal EEG, CTS may be
very small or they may appear only during non-REM sleep (3–35%)2. In serial EEGs of the
same child, CTS may occur right or left, infrequently or frequently, and appear small or giant,
alone or with spikes in other locations. The incidence of extra-rolandic spikes in rolandic
epilepsy is not precisely known but may be high when these are sought2.

Dipole EEG30-32, magnetoencephalography (MEG)33,34 and functional MRI35 studies have
demonstrated that the main negative spike component of CTS is usually modelled by a single
and stable tangential dipole source with the negative pole maximum in the central region and
the positive pole maximum in the frontal region.
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