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April 2012

Epilepsy in children | Classification and aetiology Michael Carter | ACF3, paediatrics, King’s College Hospital and UCL. April 2012. Classification. Manifestations Generalised: Arising within and rapidly engaging bilaterally distributed networks

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April 2012

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  1. Epilepsy in children |Classification and aetiologyMichael Carter | ACF3, paediatrics, King’s College Hospital and UCL April 2012

  2. Classification Manifestations • Generalised: Arising within and rapidly engaging bilaterally distributed networks • Focal: Originating within networks limited to one hemisphere • Unknown (e.g. infantile spasms) Electroclinical syndromes • Syndromic epilepsy (often categorised by age of onset, see later) • Constellations of symptoms (e.g. mesial temporal lobe sclerosis) • Non-syndromic epilepsy (e.g. post-stroke, perinatal insults etc.) Aetiology • Genetic • Structural-metabolic • Unknown

  3. Manifestations | Generalised

  4. Generalised epilepsies | Drugs and SEs Fetal valproate syndrome: neural tube defects, coloboma, limb defects. Also, cardiac defects, dysmorphic features and occasional global developmental delay

  5. Manifestations | Focal • Originate within networks limited to one hemisphere • Are described with specific reference to the symptoms involved, such as: • Subjective sensations (e.g. auras) • Motor (e.g. twitching) • Autonomic (e.g. sweating) • Dyscognitive features (e.g. impaired consciousness – note replaces “partial”) • And may: • Evolve to a bilateral convulsive seizure • (note replaces “secondarily generalised”) • Usually denote a focal lesion within the • brain, e.g. mesial temporal sclerosis, stroke etc. • The most common cause of acquired epilepsy, globally is … • (Emedicine)

  6. Manifestations | Focal T2-weighted magnetic resonance images reveal the increased signal and volume loss of the left hippocampus: mesial temporal sclerosis (Emedicine)

  7. Manifestations | Unknown For example, epilepsy syndromes in infancy Infantile spasms:

  8. Electroclinical syndromes An electroclinical syndromes is: “A complex of clinical features, signs, and symptoms that together define a distinctive, recognizable clinical disorder… Identifiable on the basis of a typical age onset, specific EEG characteristics, seizure types, and often other features which, when taken together, permit a specific diagnosis. The diagnosis in turn often has implications for treatment, management, and prognosis.”

  9. Electroclinical syndromes | Neonatal • Neonatal: benign neonatal seizures, others

  10. Electroclinical syndromes | Infancy

  11. Electroclinical syndromes | Childhood

  12. Electroclinical syndromes | Adolescence These are also known as idiopathic generalised epilepsies, and categorised by their manifestations

  13. Aetiology

  14. Investigations in children EEG • Indications: after second seizure (occasionally first seizure) • Issues: Low sensitivity (useful to support a diagnosis only) • Other: If normal, consider sleep EEG (with melatonin), photic stimulation, hyperventilation or telemetry MRI • Indications: <2 years of age, adults, focal onset, resistance to first-line medication Other • Routine biochemistry (including magnesium) and ECG in all patients

  15. Criteria for onward referral to 3ary Epilepsy nurses To be involved in the care of all children with epilepsy Syndromes and manifestations • All children with infantile spasms • All children with suspected Dravet syndrome • All children with suspected Lennox-Gastaut syndrome • All children <2 years • All children with unilateral structural lesion • All children with drug-resistant epilepsy (after 2 drugs with breakthrough) • All children with diagnostic doubt

  16. Conclusions Classification of epilepsies • By manifestation: generalised, focal, unknown • By electroclinical syndrome: usually also by typical age of onset • By aetiology: genetic, structural-metabolic, unknown Management • EEG (+/- MRI) after second seizure unless focal deficits or unwell • Epilepsy nurses • Generally: valproate first-line for any generalised epilepsy • Generally: carbamazepine (CI absences) or lamotrigine (CI myoclonus) for focal epilepsy • Importance of explaining side-effects

  17. References NICE (2012) The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. NICE Clinical Guideline 137 Berg et al. (2010) Revised terminology and concepts for organisation of seizures and the epilepsies: report of the ILAE commission on classification and terminology, 2005-2009. Epilepsia 51(4): 676-685 Panayiotopoulos (2005) The Epilepsies: seizures, syndromes and management. Bladon Medical, Oxford

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