Page 96 - ILAE_Lectures_2015

Approximately 10–20% of autonomic seizures and autonomic status epilepticus in children is
due to heterogeneous cerebral pathology4,73. These symptomatic cases are betrayed by abnormal
neurological or mental state, abnormal brain imaging and background EEG abnormalities. PS
is significantly different from rolandic epilepsy and ICOE-G, despite some overlapping clinical
and/or EEG features and these are detailed in the relevant section of this paper.

Prognosis
PS is remarkably benign in terms of its evolution4,73-80 but autonomic seizures are of concern in
the rare context of cardiorespiratory arrest4,83,5,96. The majority of patients have a single or less
than five seizures until remission. Only one-quarter have multiple and sometimes very frequent
and prolonged seizures that may be resistant to treatment. Remission often occurs within 1–2
years of onset but probably 10% may have more protracted active seizure periods. One-fifth of
patients develop rolandic and less often occipital or other seizures but these are also age related
and remit4. Atypical evolution of PS similar to those described in rolandic epilepsy is rare
probably less than 3%80,117-119.

The risk of epilepsy in adult life appears to be no higher than in the general population4,80,83.

Subtle neuropsychological deficits in some children during the active phase120 may be
syndrome-related symptoms in PS, but may also reflect effects of AEDs (most of the children
were on AEDs including phenobarbital and vigabatrin) and/or other contributing factors.
Prognosis of cognitive function is good even for patients with atypical evolutions80.

Idiopathic childhood occipital epilepsy of Gastaut

ICOE-G is a relatively rare form of pure occipital epilepsy accounting for about 2–7% of benign
childhood focal seizures2,74,76,121-130. Age at onset ranges from 3–15 years, but most frequently
it starts between 8–11 years. Both sexes are equally affected.

Clinical manifestations
Seizures are occipital and primarily manifest with elementary visual hallucinations, blindness
or both2,121-124,126,127. They are usually frequent, brief and diurnal.

Visual ictal symptoms
Elementary visual hallucinations are the commonest and most characteristic ictal symptom of
ICOE-G. They are frequently the first and often the only seizure symptom. They develop
rapidly within seconds and consist mainly of small multicoloured circular patterns that often
appear in the periphery of a visual field, becoming larger and multiplying during the course of
the seizure, frequently moving towards the other side. Ictal blindness is probably the second
most common symptom after visual hallucinations. It is sudden, usually total and it is frequently
the first and often the only seizure symptom in patients who may also have other visual seizures
without blindness. Impairment of visual awareness is consistently reported by some patients
before the appearance of visual hallucinations. Complex visual hallucinations such as faces and
figures and visual illusions such as micropsia, palinopsia and metamorphopsia occur in less
than 10% of patients and mainly after the appearance of other visual symptoms122.

Non-visual ictal occipital lobe symptoms
Non-visual occipital symptoms usually appear after the elementary visual hallucinations and
these, in order of prevalence, are deviation of the eyes, eyelid fluttering or repetitive eye
closures and sensory hallucinations of ocular movements2,121,122,124,126,127.

Deviation of the eyes, often associated with ipsilateral turning of the head, is the most common
(in about 70% of cases) non-visual ictal symptom. It usually starts after the commencement of
visual hallucinations and may be mild, but more often it is forceful tonic and may progress to