Paeds · neurology-neurodisability-and-neuromuscular
Absence, focal and generalised epilepsies
Also known as childhood absence epilepsy · juvenile myoclonic epilepsy · self-limited epilepsy with centrotemporal spikes · rolandic epilepsy · BECTS · focal epilepsy · generalised tonic-clonic epilepsy · ILAE classification of the epilepsies
A fellowship approach to absence, focal and generalised epilepsies: the child with staring spells whose EEG shows three-per-second spike-and-wave, the teenager whose morning jerks declare juvenile myoclonic epilepsy, and the ILAE 2017 framework that turns a seizure description into a syndrome, an aetiology, and a matched first-choice medicine.
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Red flags
- Never give carbamazepine or phenytoin to a child with absence or myoclonic epilepsy — they worsen both and can precipitate absence status epilepticus
- Avoid valproate in girls and women of childbearing potential wherever possible — high teratogenicity and a dose-dependent neurodevelopmental risk that the Medicines and Healthcare products Regulatory Agency restricts under the Prevent programme
- Childhood absence epilepsy that persists or first appears after ten years, or that lacks the classic three-per-second spike-and-wave, is not typical CAE — reconsider the syndrome before committing to ethosuximide
- A new focal epilepsy at any age, or a change in seizure pattern in an established epilepsy, demands neuroimaging — a structural aetiology may be operable and a tumour must not be missed
- A generalised tonic-clonic seizure in a child on treatment may be non-adherence, an intercurrent illness, or a wrong medicine for the syndrome — check the level, check the diagnosis, do not simply increase the dose
- Any developmental regression or plateau alongside new-onset epilepsy is a developmental and epileptic encephalopathy until proven otherwise and warrants urgent paediatric neurology referral
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Care settings
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- Neurology - recognise and investigate common seizure disorders and epilepsies
- Developmental and behavioural paediatrics - epilepsy and neurodevelopmental comorbidity
- Growth and Development
- Neurology - diagnose and manage childhood epilepsy syndromes and apply the ILAE classification
- Acquired and genetic neurological disease - antiseizure medicine selection and the valproate-in-pregnancy rule
- Renewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatrics
- Neurology 10-12
- Acute presentations 10-12
- Clinical Applications
- Long Cases
- Short Cases
- 9. Neurology - epilepsy and paroxysmal disorders
- 1. Neonates and infants with neurology
- 4. Professional skills and knowledge: Patient management
- Foundation of Practice (FOP)
- Theory and Science (TAS)
- Applied Knowledge in Practice (AKP)
- Clinical
- History
- Communication
- Video
- General Pediatrics Content Outline - D16 Neurology (6%)
- General Pediatrics Content Outline - Universal Task 4: Management and Treatment
- General Pediatrics Content Outline - Universal Task 2: Health Maintenance
- Patient Care 4: Clinical Reasoning
- Patient Care 5: Patient Management
- Medical Knowledge 1: Congenital anomalies and acquired disease
- Systems-Based Practice 2: Coordinated chronic disease care
- Medical Expert
- Pediatrics: Foundations EPA #8 - Communicating assessment findings and management plans to patients and families
- Pediatrics: Neurology applied domain
A seven-year-old girl is reported by her teacher to be staring blankly and not responding for ten seconds, many times a day, with rapid return to normal and no memory of the events; her EEG shows the three-per-second generalised spike-and-wave of childhood absence epilepsy. A fourteen-year-old boy presents before breakfast with a few jerks of his arms that throw his toothbrush, and the same morning a generalised tonic-clonic seizure; the combination is juvenile myoclonic epilepsy. An eight-year-old boy has nocturnal twitching of one side of the face with drooling and preserved awareness — self-limited epilepsy with centrotemporal spikes. The task in each is to classify the syndrome, confirm with EEG, image the brain where focal, and match the first-choice antiseizure medicine. [4] [6]
Run the pathway as five moves. Witness the seizure in detail from a reliable observer, because the semiology is the diagnosis. Wave is the EEG that confirms the syndrome — three-per-second spike-and-wave for absence, photic or generalised polyspike for juvenile myoclonic, centrotemporal spikes for rolandic. Weight the aetiology with MRI and genetics when the epilepsy is focal or atypical, because a structural lesion may be operable. Write the first-choice medicine for the syndrome, never carbamazepine or phenytoin for absence or myoclonus. Watch the child over years for seizure freedom, comorbid learning and behaviour, and the moment to withdraw medicine. [1] [4]
Overview & Definition
Epilepsy is a chronic disorder of the brain marked by an enduring predisposition to generate epileptic seizures, and it is defined operationally by the International League Against Epilepsy as at least two unprovoked seizures more than twenty-four hours apart. A single unprovoked seizure with a sixty per cent or greater recurrence probability over ten years also satisfies the definition, as does a recognised epilepsy syndrome. [3]
The fellowship framing rests on one distinction that governs everything that follows. A seizure is the event, while the epilepsy is the disease that produces it, and a syndrome is the pattern of seizure type, age of onset, EEG, and comorbidity that points to a cause and a treatment. Classifying the seizure is only the first step; naming the syndrome is what makes management rational. [1] [2]
The clinical importance comes from what the wrong label does to the child. An absence epilepsy labelled as focal and treated with carbamazepine worsens, and a focal epilepsy labelled as generalised and denied imaging misses an operable lesion. The ILAE 2017 framework — seizure type, then epilepsy type, then aetiology, then syndrome — is the safeguard against both errors, and it is the structure a fellowship candidate must be able to recite aloud. [1] [4]
References10ShowHide
- [1]Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia, 2017.PMID 28276062
- [2]Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia, 2017.PMID 28276060
- [3]Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official report: a practical clinical definition of epilepsy. Epilepsia, 2014.PMID 24730690
- [4]Specchio N, Wirrell EC, Scheffer IE, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia, 2022.PMID 35503717
- [5]Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia, 2022.PMID 35503712
- [6]Riney K, Bogacz A, Somerville E, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia, 2022.PMID 35503725
- [7]Glauser TA, Cnaan A, Shinnar S, Hirtz DG, Dlugos D, Masur D, Clark PO, Capparelli EV, Adamson PC; Childhood Absence Epilepsy Study Team. Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsy. N Engl J Med, 2010.PMID 20200383
- [8]Glauser T, Ben-Menachem E, Bourgeois B, et al. Updated ILAE evidence review of antiepileptic drug efficacy and effectiveness as initial monotherapy for epileptic seizures and syndromes. Epilepsia, 2013.PMID 23350722
- [9]Marson AG, Appleton R, Baker GA, et al. A randomised controlled trial examining the longer-term outcomes of standard versus new antiepileptic drugs. The SANAD trial. Health Technol Assess, 2007.PMID 17903391
- [10]Shinnar S, Cnaan A, Hu F, Clark P, Dlugos D, Hirtz DG, Glauser TA; Childhood Absence Epilepsy Study Team. Long-term outcomes of generalized tonic-clonic seizures in a childhood absence epilepsy trial. Neurology, 2015.PMID 26311751