Page 93 - ILAE_Lectures_2015

Ictal autonomic symptoms
Seizures commonly commence with autonomic manifestations (80–90%) while consciousness
and speech, as a rule, are preserved. Ictus emeticus (nausea, retching, vomiting) culminates in
vomiting in 74–82% of seizures; in others, only nausea or retching occurs and, in a quarter,
emesis may not be apparent. Emesis is usually the first apparent ictal symptom, but it may also
occur long after the onset of other manifestations. Other autonomic manifestations include
pallor (93%), incontinence of urine (19%) and faeces (3%), hypersalivation (10%), mydriasis
(7%) and less often miosis (2%), coughing and abnormalities of intestinal motility (3%).
Breathing (7%) and cardiac irregularities are rarely reported though they may be common in
mild forms. Tachycardia is a common finding, sometimes at the onset of ictal EEG75,92-94.
Cardiorespiratory arrest is rare, probably occurring in 1 per 200 individuals (four possible cases
out of around 1000 patients with PS have been reported)4,83,95. Raised temperature has been
documented in a few cases after seizure onset. Cephalic auras of discomfort and odd sensations
or headache commonly occur with other autonomic symptoms at seizure onset.

Syncope-like manifestations occur in at least one-fifth of seizures4,83,90,96. The child becomes
‘completely unresponsive and flaccid like a rag doll’ which may precede, be concurrent with
other seizure symptoms, or be the sole manifestation of a seizure4,75. They may occur while the
patient is standing, sitting, lying down or asleep and last from 1–2 minutes to half an hour195.

Ictal behavioural changes
Restlessness, agitation, terror or quietness, may appear at the onset of seizures, often in
combination with other autonomic manifestations.

Ictal non-autonomic symptoms
Pure autonomic seizures and pure autonomic status epilepticus appear to occur in 10% of
patients. They commence and terminate solely with autonomic symptoms. In all other seizures,
autonomic manifestations are followed or occasionally start with conventional seizure
symptoms. The child gradually or suddenly becomes confused and unresponsive. Unilateral
deviation of the eyes is common (60–83%), occur with or without vomiting, seldom happens at
onset and may be brief or lengthy. In some patients eyes open widely and remain in mid-
position instead of deviating to one side.

Other ictal symptoms include speech arrest (8–13%), hemifacial convulsions (6–13%), visual
hallucinations (6–10%), oro-pharyngo-laryngeal symptoms (3%), unilateral drooping of the
mouth (3%) and rarely (1%) eyelid or limb jerks, nystagmus and automatisms. The seizures
may end with hemiconvulsions often with jacksonian marching (19–30%), or generalised
convulsions (21–36%).

Duration of seizures and precipitating factors
The seizures are usually lengthy of over six minutes and almost half of them last for more than
30 minutes to many hours, thus constituting autonomic status epilepticus4,96. Lengthy seizures
are equally common in sleep and wakefulness. Even after the most severe seizures and status,
the patient is normal after a few hours’ sleep. There is no record of residual neurological
abnormalities. Hemiconvulsive or convulsive status epilepticus is rare (4%).

Two-thirds of seizures start in sleep. Many seizures have been witnessed while travelling in a
car, boat or aeroplane. The reason for this may be because in these circumstances the child
easily falls asleep, seizures are more likely to be witnessed and because travelling also
precipitates motion sickness, to which children are particularly susceptible.