Epilepsy in Children and Young People

Children and young people may present with similar types of seizures and in similar ways to adults with epilepsy (see separate article). However this article focuses on the forms of epilepsy and aspects of management which apply to children and young adults. Status epilepticus is also dealt with in a separate article.

• Epilepsy is a common neurological disorder in childhood. Seizures and epilepsy affect infants and children more than any other age group.¹
• Epilepsy is about twice as common in children as in adults (about 700 per 100,000 in children under the age of 16 years compared to 330 per 100,000 in adults).

Presenting features in children are similar to those in adults. However these sub-types are commoner, or occur exclusively, in childhood:

• Typical absences (petit mal seizures): petit mal epilepsy is manifest by frequent (as many as 100 times per day or more) episodes of brief staring spells (lasting seconds at a time):
  • Onset in childhood, and attacks continuing into adult life are rare.
  • A typical absence attack lasts only a few seconds. The onset and termination are abrupt. The child ceases what he is doing, stares, looks a little pale, and may flutter the eyelids.
  • Sometimes more extensive bodily movements occur, such as dropping the head forwards, and there may be a few clonic movements of the arms.
  • The interruption of the normal stream of consciousness is very brief, and the child may be unaware of the attacks, as indeed may be the parents for some time after onset, assuming that the child is just day-dreaming.
  • About one-third of all children with petit mal will have one or more tonic-clonic convulsions.
• Infantile spasms:
  • Occur in infants aged 4-8 months.
  • Consist of clusters of myoclonic spasms that occur when waking up.
• Juvenile myoclonic epilepsy:
  • Occurs in the teen years.
  • Early morning sudden myoclonic jerks, especially of the arms and shoulders.
  • Often later develop generalised tonic-clonic seizures.
  • May be inherited as autosomal dominant.
• Benign Rolandic Epilepsy:
  • Also known as benign partial epilepsy.
  • It occurs in children aged 4-10 years and is more common in boys.
  • Nocturnal seizures that are characterized by facial twitching and aphasia.
  • Some children with benign rolandic epilepsy may also have generalized tonic-clonic seizures.
• Benign childhood epilepsy with centrotemporal spikes:
  • Some older children may have partial or generalized seizures.
  • The inter-ictal EEG is characterized by large spike discharges over the Rolandic area of one hemisphere.
  • Is not associated with any structural lesion and has an excellent prognosis.

• Malformations: e.g. tuberous sclerosis and other hamartomas.
• Infections: meningitis and encephalitis; parasitic infections, particularly cysticercosis, are common causes of epilepsy in Third World countries.
• Electrolyte disturbances: e.g. hypernatraemia, hyponatraemia, hypoglycaemia, hypocalcaemia, hypomagnesaemia, toxins.
• Trauma.
• Metabolic defects.

Trigger factors

• Lack of sleep and watching television are two common triggers.
• It has been shown that observing the set with one eye covered prevents the occurrence of these seizures.

Non-epileptic attacks include:²

• Vasovagal syncope: loss of consciousness occurring in crowded trains, waiting at bus stops, or in school assembly; should always be presumed to be syncopal in nature unless there is clear-cut evidence to the contrary.
• Night Terrors: these affect children aged between about 6 and 8 years, who suddenly awaken from a sound sleep, wide-eyed, screaming, and inconsolable. They are amnesic for the events the following morning. They seem to occur just as often in happy children as in children who are not doing well at school or in the family. Fortunately they, too, pass quickly.
• Breath-holding attacks: affect younger children, aged between 1 and 2 years. A typical story is of a child who has some minor injury, or who is crossed in some way so that he becomes suddenly angry, upset, or frightened. Such attacks terminate spontaneously without treatment.
• Febrile convulsions
• Munchausen's by proxy
• Arrhythmias
• Migraine
• Narcolepsy

• EEG with photic stimulation and hyperventilation:
  • To support a diagnosis of epilepsy if the clinical history suggests it.
  • To help determine seizure type and epilepsy syndrome.
  • To assess the risk of seizure recurrence after a first unprovoked seizure.
  • If an EEG is necessary, it should usually be performed only after the second epileptic seizure. It should not be used to exclude a diagnosis of epilepsy or in the case of probable syncope (as there is a risk of false-positive result).
  • An interictal EEG may be normal.⁴
• Neuroimaging: to identify structural abnormalities that cause certain epilepsies. Not used routinely when a diagnosis of idiopathic generalised epilepsy has been made.
  • MRI: is the imaging investigation of choice for people with epilepsy. It is particularly important for children:
    • Who have developed epilepsy before the age of 2 years.
    • Who have any suggestion of a focal onset from history, examination or EEG (unless there is clear evidence of benign focal epilepsy).
    • In whom seizures continue in spite of first-line medication.
  • CT scan is an alternative to MRI:
    • If MRI is contraindicated or unavailable.
    • For children in whom a general anaesthetic or sedation would be required for MRI but not CT.
    • In an acute situation, to determine whether a seizure has been caused by an acute neurological lesion or illness.
• Blood tests and urine biochemistry to exclude other diagnoses and to determine an underlying cause of the epilepsy.
• ECG: if the diagnosis is uncertain.

• Provide regular structured review by a specialist at least once a year, but probably more frequently (every 3-12 months) depending on need.
• Use monotherapy whenever possible. The formulation or brand of anti-epileptic drug (AED) should not be changed (variations in bioavailability or different pharmacokinetic profiles may increase the potential for reduced effect or excessive side effects).
• Indications for monitoring AED blood levels:
  • Detection of non-adherence to the prescribed treatment.
  • Suspected toxicity.
  • Adjustment of phenytoin dose.
  • Management of pharmacokinetic interactions.
  • Specific clinical conditions (e.g. status epilepticus, organ failure or pregnancy).
  • Asymptomatic minor abnormalities in blood test results are not necessarily an indication for changes in medication.
• Withdrawing treatment:
  • When a child has been seizure-free for at least 2 years, discuss continuing or withdrawing AED treatment with the child and their families/carers, as appropriate.
  • The decision to withdraw medication should be taken by the child, their family and/or carers, as appropriate, and the specialist after a full discussion of the risks and benefits of withdrawal.
  • Withdraw gradually (over 2-3 months or longer); be aware of possible seizure recurrence. Slower withdrawal is required for benzodiazepines (6 months or longer).
  • Be aware of drug-related withdrawal symptoms and/or seizure recurrence.
  • Withdraw one drug at a time.
  • Agree with the child with epilepsy and their family and/or carers a failsafe plan of action if seizures recur (last dose reduction reversed, medical help sought).

First line anti-epileptic drugs³

• Childhood absence epilepsy: ethosuximide, lamotrigine, sodium valproate.
• Juvenile absence epilepsy: lamotrigine, sodium valproate.
• Juvenile myoclonic epilepsy: lamotrigine, sodium valproate.
• Generalised tonic-clonic seizures only: carbamazepine, lamotrigine, sodium valproate, topiramate.
• Focal epilepsies: carbamazepine, lamotrigine, oxycarbazepine, sodium valproate, topiramate.
• Infantile spasms: vigabatrin is recommended as a first-line therapy for the management of infantile spasms. Steroids are also used.
• Benign epilepsy with centrotemporal spikes: carbamazepine, lamotrigine, oxycarbazepine, sodium valproate.
• Benign epilepsy with occipital paroxysms: carbamazepine, lamotrigine, oxycarbazepine, sodium valproate.
• Severe myoclonic epilepsy of infancy: clobazam, clonazepam, sodium valproate, topiramate.
• Continuous spike wave of slow sleep: clobazam, clonazepam, ethosuximide, lamotrigine, sodium valproate, steroids.
• Lennox-Gastaut syndrome: lamotrigine, sodium valproate, topiramate.
• Landau-Kleffner syndrome: lamotrigine, sodium valproate, steroids.
• Myoclonic astatic epilepsy: clobazam, clonazepam, sodium valproate, topiramate.

Other interventions³

• Psychological interventions: consider using relaxation and cognitive behaviour therapy in children with drug-resistant focal epilepsy.
• Psychological therapies should be used as adjunctive therapy, not as an alternative to pharmacological treatment.
• Vagus nerve stimulation: an adjunctive therapy to reduce the frequency of seizures in children who are refractory to AED therapy but who are not suitable for resective surgery, including children whose epileptic disorder is dominated by partial seizures (with or without secondary generalisation) or generalised seizures.
• Ketogenic diet: consider as an adjunctive treatment in children with drug-resistant epilepsy.

Surgery

• Modern techniques for the accurate localisation of epileptic discharge and the recognition of specific seizure patterns has increased the role of surgery in the management of drug-resistant epilepsy.
• Operations include temporal lobectomy, hemispherectomies and division of the corpus collosum.

• Although for most children epilepsy is a relatively benign disorder, for some, epilepsy can be designated as "catastrophic" because the seizures are so difficult to control and because they are strongly associated with mental retardation.
• Many children with catastrophic epilepsy have the seizures as a result of underlying brain abnormalities that will inevitably lead to mental retardation whether or not they have seizures. In some patients, however, the mental retardation appears to be caused by the seizures.⁵
• Continuing epilepsy is more likely in those with neurological impairment, frequent seizures, many types of seizures or other additional medical conditions.⁶ Conditions identified by a prospective UK study included Lennox-Gastaut syndrome, Addison's disease, hearing loss, deaf mutism after meningitis, and congenital heart disease.³
• After age 16 there is a high death rate in young people with epilepsy. This emphasises the importance of maintaining supportive relationships between health care professionals and people with epilepsy as they become independent adults.
• In many children the seizures remit but it can have a major impact on a child's development and affect adult life.⁶