Key Facts

Childhood epilepsy is a heterogeneous group of conditions with varying prognoses. Accurate classification is essential as it determines treatment choice and prognosis. Many childhood epilepsy syndromes have excellent outcomes with appropriate treatment.

Epidemiology

Epilepsy affects approximately 0.5-1% of children. Incidence is highest in the first year of life. ~60% of childhood epilepsy is well controlled with monotherapy. ~30% have drug-resistant epilepsy. ~50% of childhood epilepsy achieves sustained remission by adulthood.

Aetiology

• Genetic: Idiopathic generalised epilepsies (CAE, JME, JAE), SCN1A mutations (Dravet syndrome)
• Structural: Cortical dysplasia, hippocampal sclerosis, tuberous sclerosis, brain tumour, previous stroke/injury
• Metabolic: Pyridoxine deficiency, glucose transporter deficiency (GLUT-1)
• Immune: Autoimmune encephalitis (NMDA-receptor)
• Infectious: Post-meningitis/encephalitis

Pathophysiology

Seizures result from abnormal, excessive, synchronous neuronal activity. In focal epilepsy, this arises from a localised cortical region. In generalised epilepsy, corticothalamic circuits generate bilateral synchronous discharges. The balance between excitatory (glutamate) and inhibitory (GABA) neurotransmission is disrupted.

Common Childhood Epilepsy Syndromes

Childhood Absence Epilepsy (5-7yr):

• Brief (5-15 sec) blank spells with eye fluttering
• EEG: 3Hz generalised spike-wave
• Excellent prognosis; ~70% remit by adolescence

Juvenile Myoclonic Epilepsy (12-18yr):

• Morning myoclonic jerks + absence seizures + GTC seizures
• EEG: 4-6Hz polyspike-wave
• Lifelong treatment usually required

Benign Epilepsy with Centrotemporal Spikes (BECTS/Rolandic, 3-13yr):

• Nocturnal focal seizures with face/tongue involvement, drooling
• EEG: Centrotemporal spikes
• Almost all remit by mid-adolescence; may not need treatment

West Syndrome (3-12 months):

• Infantile spasms (clusters of flexor spasms on waking)
• EEG: Hypsarrhythmia
• Treatment: Vigabatrin (if tuberous sclerosis) or prednisolone/tetracosactide

Red Flags

• Onset <1 year — higher likelihood of structural/genetic/metabolic cause
• Developmental regression with seizures — neurodegenerative disease
• Refractory seizures — consider surgical evaluation
• Infantile spasms — urgent treatment to preserve neurodevelopment

Bedside

• Detailed seizure history: Description from witness, video if available, precipitants, duration, postictal state
• Developmental assessment: Regression or delay

Bloods

• Blood glucose, calcium, magnesium, U&Es: First seizure workup
• FBC, LFTs: Baseline before starting AEDs
• Genetic testing: If epilepsy syndrome suggests genetic cause

Imaging

• MRI brain: Indicated for focal seizures, onset <2 years, developmental concerns, abnormal neurology; NOT routinely needed for typical generalised epilepsy syndromes

Special Tests

• EEG: Supports diagnosis and syndrome classification — interictal EEG may be normal (normal EEG does not exclude epilepsy)
• Sleep-deprived EEG: Increases sensitivity
• Video-EEG: For diagnostic uncertainty or presurgical evaluation
• Genetic testing: Epilepsy gene panel, chromosomal microarray — increasingly important for diagnosis and treatment selection

Non-pharmacological

• Seizure first aid education: Recovery position, timing, when to call ambulance (>5 min)
• Emergency medication plan: Buccal midazolam prescription and training for parents/school
• Lifestyle: Regular sleep, avoid known triggers; swimming with supervision; driving regulations (DVLA — 1 year seizure-free for driving)
• EHCP: Educational support if needed

Pharmacological (NICE NG217)

Focal seizures:

• First-line: Carbamazepine or lamotrigine
• Second-line: Levetiracetam, oxcarbazepine, sodium valproate

Generalised tonic-clonic:

• First-line: Sodium valproate (males) or lamotrigine (females of childbearing potential)
• Second-line: Levetiracetam, clobazam

Absence seizures:

• First-line: Ethosuximide or sodium valproate
• Second-line: Lamotrigine

Myoclonic seizures:

• First-line: Sodium valproate (males) or levetiracetam
• AVOID carbamazepine (worsens myoclonic/absence seizures)

Infantile spasms:

• Vigabatrin (if tuberous sclerosis) or prednisolone/tetracosactide

Surgical/Interventional

• Epilepsy surgery: For drug-resistant focal epilepsy with identifiable lesion — resective surgery; ~60-70% seizure-free
• Vagus nerve stimulation (VNS): Adjunctive for drug-resistant epilepsy
• Ketogenic diet: Evidence-based for drug-resistant epilepsy; particularly effective in GLUT-1 deficiency and some genetic epilepsies

Referral Criteria

• All first seizures — paediatric assessment
• Drug-resistant epilepsy (failure of 2 appropriate AEDs) — tertiary epilepsy centre
• Infantile spasms — urgent paediatric neurology
• Consideration of epilepsy surgery — specialist epilepsy centre

• ~60% achieve seizure freedom with first AED
• ~50% achieve sustained remission by adulthood
• BECTS: Almost 100% remission by mid-adolescence
• CAE: ~70% remit by adolescence
• JME: Lifelong treatment usually required; well controlled in most
• Drug-resistant epilepsy: ~30%; some benefit from surgery
• SUDEP: Risk ~1 per 1,000 patient-years; higher with uncontrolled GTC seizures

AED Choice by Seizure Type (NICE NG217)

Status Epilepticus Protocol (APLS)