Classification of Epilepsy in Children

1. Classification of Epilepsy in Children Dr Tina Williams PLEAT Frimley Park Hospital June 2011

2. Epilepsy • Condition with recurrent, unprovoked seizures • Old Classification : ILAE 1989 Partial (Simple or Complex) and Generalised

3. ILAE 2001 – More than just classification • Axes 1 to 4* • More than just identifying seizure type • Attempting to identify a clear Epilepsy Syndrome • Specific Treatment, Prognosis

4. Take Away today....... Think Axes: • Description – Sz or not, videos • Seizure Type • Features of Identifiable Syndrome? • Specific Rx – NICE 2004, BNFc

5. Axis 1- Ictal phenomenology • Description of episodes – Signs and Symptoms, Standardised Terminology • Video recordings • Events related

6. Definitions • Focal (previously ‘partial’) seizure - initial activation of only part of one cerebral hemisphere occurs (although may generalize). (Luders 2001) • Generalized seizure – discharge from both cerebral hemispheres occurs. Loss of Consciousness may occur (Luders 2001)

7. Axis 2 Seizure Types

8. I Self Limited • Focal • Generalised • II Continuous (status epilepticus) • Focal • Generalised

9. Self-limited seizure types I Generalized seizures • Tonic-clonic seizures • Clonic seizures • Typical absence seizures • Atypical absence seizures • Tonic seizures • Myoclonic seizures • Atonic seizures II Focal seizures • Focal sensory seizures • Focal motor seizures • (tonic/clonic/myoclonic seizures) • With typical automatisms (Complex Partial Seizures) III Secondarily Generalized seizures

10. Origin of symptoms and signs in focal seizures - Visual display over the dominant hemispheres

11. Continuous Seizure Types I I Generalized status epilepticus II Focal status epilepticus • Epilepsiapartialis continua • Aura continua • Hemiconvulsive status with hemiparesis

12. Axis 3 Epilepsy Syndromes

13. Epilepsy Syndromes • An epileptic disorder or condition characterised by cluster of signs and symptoms customarily occurring together. • List not exhaustive

14. Epilepsy Syndromes Benign • Idiopathic focal epilepsies of infancy and childhood • Familial focal epilepsies (autosomal dominant) • Idiopathic generalized epilepsies Malignant • Symptomatic focal epilepsies (Focal Pathology eg tumour, bleed, infarct) • Epileptic encephalopathies

15. Epilepsy Syndromes – Benign I  Idiopathic focal epilepsies of infancy and childhood • Benign infantile seizures • Benign childhood epilepsy

16. Epilepsy Syndromes – Benign II Familial focal epilepsies (autosomal dominant) • Benign familial neonatal seizures • Benign familial infantile seizures • Autosomal dominant nocturnal frontal lobe epilepsy

17. Epilepsy Syndromes – Benign III Idiopathic generalized epilepsies • Benign myoclonic epilepsy in infancy • Childhood absence epilepsy • Epilepsy with generalized tonic-clonic seizures only

18. Epilepsy Syndromes - Malignant Epileptic encephalopathies • Early myoclonic encephalopathy • West syndrome • Lennox-Gaustaut syndrome • Landau-Kleffner syndrome

19. Axis 4 Aetiology or Underlying Cause

20. Aetiology / Disease Causing Epilepsy • Neurocutaneous Disorders • Malformations due to abnormal cortical developments • Other cerebral malformations • Tumours • Bleeds/ Infarcts • Chromosomal abnormalities • Inherited metabolic disorders • Pre/ perinatalischaemic/ anoxic lesions or cerebral infections • Postnatal infections

21. Common Syndromes

22. Benign Epilepsy of Childhood With Centrotemporal Spikes • Benign Rolandic Epilepsy • Idiopathic, otherwise healthy children. • EEG - high-voltage centrotemporal spikes often followed by a slow wave. • Onset usually 4-11yrs, peaks at 5-9yrs • Boys:Girls - 6:4 • Unilateral somatosensory aura, Speech arrest, conscious in most cases • Secondary generalisation: tonic/T-C common • May be nocturnal • Rx – Carbamazepine usually • Prognosis good

23. Benign Myoclonic Epilepsy in Infancy • 30% have a family Hx of Epilepsy • Onset from 6 months – 3 years of age • No other seizure types • Usually upper extremities and head • EEG may be normal, sleep EEG may show changes. • Prognosis : Good, up to 50% may have developmental/ language delay

24. Childhood Abscence Epilepsy • Onset 4-10 yrs; Peak 5-7yrs • Female > Male • Mild automatisms frequent, but major motor involvement diagnosis. • The EEG - characteristic "typical 3Hz spike-wave" discharges. • Prognosis is excellent in well-defined cases of CAE with most patients "growing out" of their epilepsy

25. Juvenile Abscence Epilepsy • Onset 10-17 years, peak 10-12 years • Male=Female • More sporadic than CAE • > 75% have tonic-clonic seizures • EEG - spike-wave discharges most prominent in the frontal region. Faster (3.5 Hz to 4.5 Hz) than in typical childhood absence epilepsy. • Prognosis: Respond well to Rx – Valproate, Ethosuxamide. If no other factors, prognosis good.

26. West Syndrome • Usually abnormal brain eg TS - Invx • Triad: infantile spasms, EEG pattern termed hypsarrhythmia, and mental retardation • Spasms affecting head and upper extremities lasting 5-20seconds, clustering, sleep times • Rx: ACTH/Steroids/ Vigabatrin • Prognosis – Seizure control often. Developmental delay progresses

27. Lennox Gastaut Syndrome • Childhood Epileptic Encephalopathy • 1-4% of childhood epilepsies • Multiple sz types, Dev Delay/regression often follows • EEG: Gen slow spike+wave discharges • Common sz: tonic-axial, drops, atypical absences, but can be myoclonic, gen tonic-clonic, focal. • Often resistant to Rx. • Surgery to remove corpus callosum/ lobectomy works for select grous • Ketogenic diet works in some

28. Landau-Kleffner Syndrome • Onset 3-7 yrs • Rare disorder • Loss of expressive language → loss of speech • Rx – Speech Rx, AED • Prognosis: Variable, Age of onset after 6yrs is better

29. Rett Syndrome • Females • Loss of skills – speech, purposeful hand movements • Develop stereotypic Hand movements • Onset 3months-3 yrs • Prognosis poor - Regression

30. Dravet’s Syndrome • Severe Myoclonic Epilepsy of Infancy • Begins in 1st year of life • Febrile seizures, status then become afebrile • Can be generalized, myoclonic, atypical abscences, clonic, tonic-clonic, or focal • EEGs - generalized and focal and multifocal anomalies • Rx – Difficult control • Prognosis – Poor neurological outcome. 50% severe

31. Summary Think Axes: • Description – Sz or not, videos • Seizure Type • Features of Identifiable Syndrome? • Specific Rx – NICE 2004, BNFc

32. Questions?