Epilepsia en niños y jóvenes

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

Epileptic seizures and epilepsy syndromes should be classified according to the description of seizure, the seizure type, the epilepsy syndrome and the aetiology. The seizure type(s) and epilepsy syndrome, aetiology, and comorbidity should be determined, because failure to classify the epilepsy syndrome correctly can lead to inappropriate treatment and persistence of seizures. Children and young people with epilepsy and/or their family/carers should be given information about their seizure type(s) and epilepsy syndrome, and the likely prognosis.¹

Children and young people with learning disabilities¹

Prevalencia

• Epilepsy is a common neurological disorder in childhood. Seizures and epilepsy affect infants and children more than any other age group.²

• Epilepsy is about twice as common in children as in adults.

• The incidence of status epilepticus in developed countries is between 17 and 23/100,000 with a higher incidence in younger children.³

• There is an increased risk of seizures in children of parents with epilepsy. The probability that a child will be affected is generally low but will depend on the family history.¹

Causes of epilepsy in children

• Malformations - eg, tuberous sclerosis and other hamartomas.

• Infections: meningitis and encephalitis; parasitic infections, particularly cysticercosis, are common causes of epilepsy in developing countries.

• Electrolyte disturbances - eg, hypernatraemia, hyponatraemia, hypoglycaemia, hypocalcaemia, hypomagnesaemia, toxins.

• Trauma.

• Trastornos metabólicos.

Trigger factors

• Watching television and lack of sleep are two common triggers.

• It has been shown that observing the set with one eye covered prevents the occurrence of these seizures.

Presentación

When a child or young person presents with a seizure, take a detailed history. If possible, use eyewitness accounts and video footage of the seizure to inform the assessment. A thorough physical examination should be performed, including cardiac, neurological and mental state. An assessment of development is important for children presenting with a seizure.¹

Presenting features in children are similar to those in adults. However, these subtypes are more common, or occur exclusively, in childhood:

• Typical absences ('petit mal' seizures):⁴

  • Frequent (as many as 100 times per day or more) episodes of brief staring spells (lasting seconds at a time).

  • Onset in childhood; attacks continuing into adult life are rare.

  • About 10% of seizures in children with epilepsy are typical absence seizures.

  • A typical absence attack lasts only a few seconds. The onset and termination are abrupt. The child ceases what he or she 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.

  • When typical absence seizures are the only type of seizures, seizures generally cease spontaneously by 12 years of age or sooner. Fewer than 10% of children develop infrequent generalised tonic clonic seizures, and it is rare for them to continue having absence seizures.

• Infantile spasms:
  

  • Occur in infants aged 4-8 months.

  • Consist of clusters of myoclonic spasms that occur when waking up.

• Dravet's syndrome:^{5 6}
  

  • Dravet's syndrome includes severe myoclonic epilepsy of infancy with the onset of recurrent febrile and/or afebrile hemiclonic or generalised seizures, or status epilepticus, in a previously healthy infant.

  • It is considered that infants who are 2-15 months old, presenting with either a first prolonged hemiclonic seizure or first convulsive status epilepticus with fever or following vaccination, in the absence of another cause, should undergo genetic testing for Dravet's syndrome.

  • Multiple seizure types develop which are generally resistant to AEDs. There is also developmental arrest or regression.

  • The prevalence of autism in studies ranges from 22% to 46%. However, autism appears to be under-recognised. Behavioural comorbidities may also be underdiagnosed and untreated, despite their significant negative impact on quality of life.

  • Mortality may be up to 15% by age 20 years.

• Síndrome de Lennox-Gastaut.

• Juvenile myoclonic epilepsy:⁷
  

  • Occurs in the teen years.

  • Early morning sudden myoclonic jerks, especially of the arms and shoulders.

  • Often later develop generalised tonic-clonic (GTC) seizures.

  • Of the three common seizure types, myoclonic, generalised tonic-clonic and absences, absences also occur independently.

  • May be inherited as autosomal dominant.

• Panayiotopoulos syndrome:⁸
  

  • Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder.

  • Affects otherwise normal children with onset at around 3-6 years.

  • Seizures are often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting.

  • Electroencephalogram (EEG) shows shifting and/or multiple foci, often with occipital dominance.

  • Often confused with occipital epilepsy and acute non-epileptic disorders - eg, encephalitis, syncope, cyclical vomiting or atypical migraine.

• Benign epilepsy with centrotemporal spikes (BECTS):⁹
  

  • Also known as self-limited epilepsy with centrotemporal spikes.

  • Mild end of epilepsy aphasia spectrum and is characterised by normal cognitive function but some cases can present with neuropsychological impairment and problems with cognition.

  • BECTS is the most common epilepsy syndrome in children.

  • Seizures originate in the Rolandic area of the brain (situated around the central sulcus of the brain, also called the centrotemporal area, located around the Rolandic fissure).

  • It affects children below 15 years with a male predominance.

  • BECTS can evolve to an atypical form known as atypical benign partial epilepsy of childhood, which is characterised by an earlier age of onset of seizures which are more severe and associated with learning, cognitive, and behavioural problems.

  • BECTS is not associated with any structural lesion and has an excellent prognosis, with most children becoming seizure-free by puberty.

Diagnóstico diferencial

See also the articles on Febrile Convulsions, Non-epileptic Seizures and Reflexic Anoxic Seizures.

Non-epileptic attacks include:¹

• 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.

• Reflexic anoxic seizures: 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 or she becomes suddenly angry, upset or frightened. Such attacks terminate spontaneously without treatment.

• Febrile convulsions.

• Fabricated or induced illness by carers.

• Arritmias cardíacas.

• Migraña.

• Apnea del sueño.

• Narcolepsia.

Remisión¹

• Refer children, young people and adults urgently (for an appointment within two weeks) for an assessment after a first suspected seizure. Refer to a paediatrician with expertise in assessing first seizures and diagnosing epilepsy.

• Referral after remission

• Refer children, young people and adults urgently (for an appointment within two weeks) for an assessment if they have a seizure recurrence after a period of remission.

Assessing the risk of a second seizure:

• When a child, young person or adult presents with a first seizure, carry out an individualised assessment of their risk of a second seizure.

• Children presenting with a first afebrile seizure (seizure without a fever) are at an increased risk of further afebrile seizures, especially within 6 to 12 months, compared with children with a febrile seizure (seizure with a fever).

• Children presenting with complicated febrile seizures (febrile seizures that last longer than 10 minutes or febrile seizures associated with other features, such as weakness, on one side of the body) may be at higher risk of epilepsy, especially if other predisposing risk factors for epilepsy are present.

Referral to a tertiary epilepsy service

Referral to a tertiary epilepsy service, to be seen within four weeks, is indicated if any of the following apply:

• Uncertainty about the diagnosis or cause of epilepsy, the seizure type or epilepsy syndrome.

• The person has an epilepsy syndrome likely to be drug-resistant, their seizures are drug-resistant or their treatment is associated with intolerable side-effects.

• Further assessment and treatment approaches are indicated, such as: video electroencephalogram (EEG) telemetry, neuropsychology or neuropsychiatry, specialised neuroimaging, specialised treatments (eg, medication that can only be prescribed by a tertiary epilepsy service or a ketogenic diet), epilepsy surgery or vagus nerve stimulation.

• The person is eligible for and wishes to participate in a clinical trial or research study.

Refer children with suspected or confirmed epilepsy to a tertiary paediatric epilepsy service to be seen within two weeks, if they:

• Are aged under 3 years.

• Are aged under 4 years and have myoclonic seizures.

• Have a unilateral structural lesion.

• Are showing deterioration in their behaviour, speech or learning.

Refer girls and young women with epilepsy who are planning pregnancy or are pregnant to an epilepsy specialist team for a review of their antiseizure medication options.

Investigación¹

• After a first suspected seizure, a 12-lead ECG should be performed to help identify cardiac-related conditions that could mimic an epileptic seizure.

• Metabolic disturbance, including hypoglycaemia, can result in seizures.

• Brain neuroimaging tests should be offered if an underlying structural cause is suspected.

Electroencefalograma (EEG)

• If the history and examination suggest an epileptic seizure, and a diagnosis of epilepsy is suspected, a routine EEG carried out while awake to support diagnosis and provide information about seizure type or epilepsy syndrome should be considered. EEG should not be used to exclude a diagnosis of epilepsy.

• Si se solicita un EEG tras una primera crisis, debe realizarse lo antes posible (idealmente en las 72 horas siguientes a la crisis).

• Provoking manoeuvres during EEG, such as hyperventilation and photic stimulation should be discussed and performed if agreed.

• If routine EEG is normal, a sleep-deprived EEG should be performed if agreed.

• If routine and sleep-deprived EEG results are normal and diagnostic uncertainty persists, ambulatory EEG (for up to 48 hours) should be considered.

Neuroimaging

• MRI scan if diagnosed with epilepsy, unless idiopathic generalised epilepsy or self-limited epilepsy with centrotemporal spikes. The MRI should be carried out within six weeks of the MRI referral. If MRI is contra-indicated, consider a CT scan.

• An additional MRI scan should be considered if the original scan was suboptimal, there are new features to their epilepsy, they have idiopathic generalised epilepsy or self-limited epilepsy with centrotemporal spikes that has not responded to first-line treatment, or surgery is being considered.

Pruebas genéticas

• Discuss with a neurologist or geneticist any uncertainties about whether to offer genetic testing or which tests to offer to a person with epilepsy. Refer to the NHS National Genomic Test Directory for rare and inherited disease for information on genetic tests commissioned by the NHS in England.

• Consider whole-genome sequencing for people with epilepsy of unknown cause who:

  • Were aged under 2 years when epilepsy started; or

  • Have clinical features suggestive of a specific genetic epilepsy syndrome (eg, Dravet's syndrome).

  • Have additional clinical features such as a learning disability, autism spectrum disorder, a structural abnormality (eg, dysmorphism or congenital malformation), or unexplained cognitive or memory decline.

• Consider whole-genome sequencing for people with epilepsy of unknown cause who were aged between 2 and 3 years when epilepsy started.

Pruebas de anticuerpos

Consider antibody testing in discussion with a neurologist for people with new-onset epilepsy if autoimmune encephalitis is suspected.

Neuropsychological assessment

La evaluación neuropsicológica debe considerarse cuando es importante evaluar las dificultades de aprendizaje y la disfunción cognitiva, en particular en lo que se refiere al lenguaje y la memoria. Está indicada la derivación para una evaluación neuropsicológica:

• When the child or young person with epilepsy is having educational difficulties.

• Cuando una resonancia magnética ha identificado anomalías en regiones cerebrales importantes desde el punto de vista cognitivo.

• Cuando se notifican déficits de memoria u otros déficits cognitivos y/o deterioro cognitivo.

Epilepsy treatment in children (management)¹

See also the separate articles on:

• Anticonvulsants used for Generalised Seizures.

• Anticonvulsants used for Focal Seizures.

• Epilepsy and Pregnancy.

• Epilepsy in Adults.

• Status Epilepticus Management.

• Fetal Anticonvulsant Syndrome.

Start treatment with an antiseizure medication once the diagnosis of epilepsy is confirmed.
Consider starting treatment after a first unprovoked seizure if any of the following apply:

• An examination identifies signs of neurological deficit.

• The electroencephalogram (EEG) shows unequivocal epileptic activity.

• After a discussion of the risk of further seizures, the person or their family or carers consider the risk unacceptable.

• Las imágenes cerebrales muestran una anomalía estructural.

A single antiseizure medication (monotherapy) should be used to treat epilepsy whenever possible. The diagnosis of epilepsy should be reviewed if seizures continue despite an optimal dose of a first-line antiseizure medication.

If first-line monotherapy is unsuccessful and epilepsy diagnosis remains confirmed, try monotherapy with another antiseizure medication, using caution during the changeover period:

• Increase the dose of the second medicine slowly while maintaining the dose of the first medicine.

• If the second medicine is successful, slowly taper off the dose of the first medicine.

• If the second medicine is unsuccessful, slowly taper off the dose of the second medicine and consider an alternative.

If monotherapy is unsuccessful, consider trying an add-on treatment:

• When starting an add-on treatment, carefully titrate the additional medicine and review treatment frequently, including monitoring for adverse effects such as sedation.

• If trials of add-on treatment do not result in a reduction in seizures, use the regimen that provides the best balance between effectiveness and
  tolerability of side effects.

Safety considerations:

Always consider the risks and potential side effects of any antiepileptic medication by referral to the BNF.¹⁰ The National Institute for Health and Care Excellence (NICE) guideline particularly highlights:

• Phenytoin is associated with an increased risk of serious skin reactions in people of Han Chinese or Thai family background.

• Carbamazepine and potentially medicines with a similar chemical structure (such as oxcarbazepine and eslicarbazepine acetate) are associated with an increased risk of serious skin reactions in people of Han Chinese, Thai, European or Japanese family background.

• Long-term treatment with some antiseizure medications (such as carbamazepine, phenytoin, primidone and sodium valproate) is associated with decreased bone mineral density and increased risk of osteomalacia.

Antiseizure medications for young women:

• Discuss with women and girls with epilepsy who are able to have children (including young girls who are likely to need treatment when they are able to have children), and their families or carers if appropriate, the risks to an unborn child of taking antiseizure medications during pregnancy, such as congenital malformations, neurodevelopmental impairments and fetal growth restriction.

• Specifically, discuss the risks to the unborn child of using sodium valproate during pregnancy, including the increased risk with higher doses and polytherapy.

• Some antiseizure medications - eg, carbamazepine, oxcarbazepine, phenytoin and topiramate - can impair the effectiveness of hormonal contraceptives.

• Oestrogen-containing hormonal contraceptives and hormone replacement therapy can impair the effectiveness of lamotrigine.

• Breastfeeding when taking antiseizure medications is generally safe and should be encouraged.

• Consider more frequent monitoring reviews for women and girls with epilepsy who are pregnant and are prescribed antiseizure medication, if they:

  • Tener una discapacidad de aprendizaje.

  • Are aged under 16 years.

  • Have active epilepsy (a seizure within the past 12 months).

  • Tiene convulsiones tónico-clónicas bilaterales.

  • Have modifiable risk factors for sudden unexpected death in epilepsy (SUDEP).

• Consider monitoring antiseizure medication levels in women or girls with epilepsy who are planning pregnancy and are considered to be at risk of their seizures worsening.

Monitoring and review:

• Agree a frequency for regular reviews that is individually tailored to the child or young person's needs, preferences and the nature of their epilepsy and at least every 12 months.

• Consider monitoring antiseizure medication levels in people with epilepsy and any of the following:
  

  • Convulsiones incontroladas.

  • Side effects from their medication.

  • A specific clinical condition needing closer supervision (such as pregnancy or renal failure).

  • Poor adherence to medication.

Discontinuing antiseizure medication:

• After a person has been seizure-free for 2 years, carry out an individualised assessment to determine the risk of seizure recurrence if antiseizure medications are discontinued. This should be carried out by an epilepsy specialist if there is any doubt or concern about the risks.

• Discuss with the person with epilepsy, and their family or carers if appropriate:

• Their individualised risk assessment, including their risk of seizures recurring and, if appropriate, the risk of SUDEP

• The person's preferences and lifestyle, including the implications for driving if relevant.

• If a decision is made to discontinue antiseizure medication, the plan should include reducing their antiseizure medications gradually:

  • For most medicines, this would typically be over at least three months.

  • For benzodiazepines and barbiturates, this would typically be over a longer period to reduce the risk of drug-related withdrawal symptoms.

• For people with epilepsy taking multiple antiseizure medications, discontinue their medications one at a time.

• If seizures recur during or after discontinuation, reverse the last dose reduction and seek guidance from the epilepsy specialist, in line with the agreed plan.

• After epilepsy surgery, discontinue antiseizure medications under the guidance of the epilepsy surgery centre.

Cannabis-based medicinal products¹³

NICE has issued technological appraisal guidance on the use of cannabidiol with clobazam for the treatment of seizures related to Dravet's syndrome. and Lennox-Gastaut syndrome.

For both syndromes, cannabidiol with clobazam is recommended as an option for treating seizures in people aged 2 years and older, only if:

• The frequency of drop seizures is checked every six months, and cannabidiol is stopped if the frequency has not fallen by at least 30% compared with the six months before starting treatment.

• The company provides cannabidiol according to the commercial arrangement.

See also the separate article on Cannabis-based Medicinal Products.

Tratamientos no farmacológicos

Ketogenic diet
A ketogenic diet, under the guidance of a tertiary epilepsy specialist, should be considered in people with:

• Certain childhood-onset epilepsy syndromes, eg: glucose transporter type 1 deficiency syndrome (GLUT1 deficiency syndrome), epilepsy associated with pyruvate dehydrogenase deficiency, infantile spasms syndrome, epilepsy with myoclonic-atonic seizures (Doose syndrome), Dravet's syndrome and Lennox–Gastaut syndrome.

• Drug-resistant epilepsy if other treatment options have been unsuccessful or are not appropriate.

Resective epilepsy surgery
Referral for resective epilepsy surgery assessment should be considered for drug-resistant epilepsy. For people with MRI abnormalities that indicate a high risk of drug-resistant epilepsy, early referral to a tertiary epilepsy service should be considered for assessment.

Vagus nerve stimulation
If resective epilepsy surgery is not suitable, vagus nerve stimulation should be considered as an add-on treatment to antiseizure medication.

Epilepsy surgery is an effective way to control seizures in patients with drug-resistant focal epilepsy, often leading to improvements in cognition, behaviour and quality of life.^{14 15 16}

• Modern techniques for the accurate localisation of epileptic discharge and the recognition of specific seizure patterns have increased the role of surgery in the management of drug-resistant epilepsy.¹⁵

• Las intervenciones quirúrgicas para la epilepsia incluyen la resección temporal anteromedial (la operación más frecuente para la epilepsia del lóbulo temporal medial), la callosotomía (para los síndromes de epilepsia generalizada), la hemisferectomía funcional y la transección subpial múltiple.

Childhood-onset epilepsies

See also the article on Lennox–Gastaut syndrome and our information on infantile spasms syndrome.

Dravet's syndrome

• Sodium valproate should be considered as first-line treatment. Sodium valproate should be used with caution in women and girls, but it is recommended as first-line treatment because of the severity of the syndrome and the lack of evidence for other effective first-line treatment options.

• If sodium valproate alone is unsuccessful as first-line monotherapy, triple therapy with stiripentol and clobazam as first-line add-on therapy should be considered.

• If triple therapy is unsuccessful and the child is over 2 years, cannabidiol in combination with clobazam as a second-line add-on treatment option should be considered.

• If triple therapy is unsuccessful for Dravet's syndrome in a child aged under 2 years or second-line treatment is unsuccessful in a child aged over 2 years, ketogenic diet, levetiracetam or topiramate should be considered.

• If all other treatment options are unsuccessful, potassium bromide under the guidance of a neurologist with expertise in epilepsy should be considered.

Self-limited epilepsy with centrotemporal spikes

• Lamotrigine or levetiracetam should be considered as first-line treatment.

• If first-line treatments are unsuccessful, second-line monotherapy options include carbamazepine, oxcarbazepine or zonisamide.

• If second-line treatments tried are unsuccessful sulthiame as monotherapy or add-on treatment should be considered, but only after discussion with a tertiary paediatric neurologist.

Epilepsy with myoclonic-atonic seizures (Doose syndrome)

• Levetiracetam or sodium valproate should be considered as first-line treatments for epilepsy with myoclonic-atonic seizures.

• If first-line treatments are unsuccessful, a ketogenic diet should be considered as a second-line monotherapy or add-on treatment, under the supervision of a ketogenic diet team.

• If second-line treatment is unsuccessful, third-line monotherapy or add-on options are clobazam, ethosuximide, topiramate or zonisamide.

• Consider discontinuing antiseizure medication treatment when seizure-free for two years.

Consulte¹

• Children and young people with epilepsy should have a regular structured review and be registered with a general medical practice.

• Children and young people should have a regular structured review with a specialist.

• El intervalo máximo entre revisiones debe ser de un año, pero la frecuencia de la revisión vendrá determinada por la epilepsia del paciente y sus deseos. El intervalo suele oscilar entre 3 y 12 meses.

• El tratamiento debe revisarse a intervalos regulares para garantizar que no se mantiene al paciente durante largos periodos con un tratamiento ineficaz o mal tolerado y que se mantiene la concordancia con la medicación prescrita.

• La revisión anual debe incluir una pregunta sobre los efectos secundarios y una discusión del plan de tratamiento para garantizar la concordancia y el cumplimiento de la medicación.

• At the review, there should be access to written and visual information, counselling services, information about voluntary organisations, epilepsy specialist nurses, appropriate investigations and referral to tertiary services including surgery, when indicated.

Transition to adults' epilepsy services¹

Ensure transition from children's to adults' epilepsy services is individually tailored to the young person with epilepsy. Begin planning transition early for young people who have complex or additional health and social care needs - eg, young people whose seizures are not yet controlled or those with learning disabilities.

During transition of young people with epilepsy to adult services, the paediatric and adult multidisciplinary teams should jointly review the person's diagnosis and management plan, taking a person-centred approach that involves the young person, and their family or carers as appropriate, in planning and decisions about their care.

Pronóstico

Although overall mortality in children with epilepsy is higher than would be expected, epilepsy-related death was rare and similar to the expected mortality in the general population. Most children died of complications of the underlying neurological disease or unrelated disease rather than as a result of epilepsy.¹⁷

• 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 general learning disability.

• Continuing epilepsy is more likely in those with neurological impairment, frequent seizures, many types of seizures or other additional medical conditions.

• Conditions associated with epilepsy identified by a prospective UK study included Lennox-Gastaut syndrome, Addison's disease, hearing loss, deaf mutism after meningitis, and congenital heart disease.¹

• The increased death rate in young people with epilepsy. This emphasises the importance of maintaining supportive relationships between healthcare professionals and people with epilepsy as they become independent adults.

• In many children the seizures remit but can have a major impact on a child's education and development and therefore affect adult life.