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an age-related maturational instability of the lower rolandic (somatosensory) cortex that
represents the face and the oropharynx bilaterally7.

Evolution and prognosis
The prognosis for rolandic seizures is invariably excellent, with probably less than 2% risk of
developing absence seizures and less often GTCS in adult life2,20,38,58-62. Remission occurs
within 2–4 years from onset and before the age of 16 years. The total number of seizures is low,
the majority of patients having fewer than 10 seizures; 10–20% have just a single seizure. About
10–20% may have frequent seizures, but these also remit with age.

Children with rolandic seizures may develop usually mild and reversible linguistic, cognitive
and behavioural abnormalities during the active phase of the disease63-68. These may be worse
in children with onset of seizures before eight years of age, high rate of occurrence and
multifocal EEG spikes69-71. The effect of antiepileptic drugs (AEDs), the impact of stigmatising
because of epilepsy, bias in selection of the most serious cases and other factors have not been
excluded in most of these studies. The development, social adaptation and occupations of adults
with a previous history of rolandic seizures was found normal58,59.

Rarely (<1%) rolandic epilepsy may evolve to more severe syndromes with linguistic,
behavioural and neuropsychological deficits, such as Landau-Kleffner syndrome, atypical focal
epilepsy of childhood or epilepsy with continuous spike and wave during sleep (CSWS)25, as
explained later in this assessment.

Panayiotopoulos syndrome

Panayiotopoulos syndrome (PS) is a common, childhood-related, susceptibility to autonomic
seizures confirmed in long-term studies of over 1000 children worldwide4,72-82. PS is defined as
‘benign age-related focal seizure disorder occurring in early and mid-childhood. It is
characterised by seizures, often prolonged, with predominantly autonomic symptoms, and by
an EEG that shows shifting and/or multiple foci, often with occipital predominance’83. ‘Early
onset benign childhood occipital epilepsy’, often used as synonymous with PS1,84, does not
represent the wide clinical, EEG and pathophysiological spectrum of PS which is far beyond
the occipital neocortex85.

Onset is from age 1–14 years with 76% starting between 3–6 years. Both sexes are equally
affected. Prevalence of PS may be high, though this is practically absent in designed controlled
epidemiological studies16,86-89 which is understandable as this syndrome was only recently
formally recognised, its features imitate many other conditions, and it often manifests with a
single seizure only. In the original cohort of Panayiotopoulos in 1988, prevalence was around
13% in children aged 3–6 years with one or more non-febrile seizures, and 6% in the age group
1–15 years. These figures may be higher if children who are currently considered to have
atypical clinical presentation are included in the syndrome4,90. A recent study found that PS is
the most common specific cause of non-febrile status epilepticus in childhood91.

Clinical manifestations
The hallmark of PS is ictal autonomic aberrations that may involve any function of the
autonomic system and mainly emesis (70–80% of seizures). The following description of
clinical manifestations of PS is based on a synthetic analysis of available clinical historical data
as perceived by patients and witnessed by observers from our records and those provided in the
literature4. Therefore, they may not accurately represent their true prevalence and sequence in
PS.