Funny Turns in Children

1. Funny Turns in Children Robert Jones, Paediatrician Andrew Smith, Senior Neurophysiology Technician

2. Learning Objectives • Develop a systematic approach to the child with funny turns • Understand the ILEA 2001 Classification • Cope with diagnostic uncertainty • Understand EEG basics • Learn from case examples with video-EEG demonstration

3. Key Questions for any child with funny turns, including those with a diagnosis of epilepsy • Is it epilepsy? • What type of seizure is it? • Is there an epilepsy syndrome? • Is there an underlying aetiology? • What is the correct management?

4. Some definitions • Epileptic Seizure-a sudden change in the electrical activity of the brain, accompanied by objective or subjective change in behaviour • Epilepsy-two or more epileptic seizures, or one episode of status epilepticus • Syndrome-a cluster of signs and symptoms, which may include evidence from clinical(eg.seizure type, neurological findings), neurophysiological and neuroradiological investigation • Semiology-the study of linguistic signs and symbols

5. ILEA Classification (Epilepsia,42(6),1-8,2001 • Five axis system- • Description of ictal semiology • Epileptic seizure type or types • Syndrome diagnosis • Specific aetiology if known • Optional designation of degree of impairment caused by condition

6. Questions Is it epilepsy? Seizure type? Syndrome? Aetiology? Management? ILEA Semiology(description!) Seizure type classified Syndrome diagnosis Aetiology Impairment (optional) Key questions and ILEA compared

7. Axis 1 – Ictal phenomenology • From the glossary of descriptive terminology, now standardised • Differing degrees of detail possible • Detail needed varies eg. candidate for surgery, clinical, research

8. Axis 2 – Seizure type • From ILEA list of epileptic seizures • Include localisation within brain if possible • Include precipitating stimuli for the reflex epilepsies if possible

9. Axis 2 – Seizure type Self limiting seizures • Generalised – tonic-clonic - clonic - myoclonic - typical absence etc • Focal - sensory - motor • Gelastic seizures • Hemiclonic seizures • Secondary generalised seizures

10. Axis 2 – Seizure type Continuous seizure types • Generalised status epilepticus - tonic-clonic - absence - myoclonic etc. • Focal status epilepticus - epilepsia partialis cont. - hemiconvulsive status - psychomotor status

11. Axis 2 – seizure type Precipitating stimuli for reflex epilepsies • Visual eg flicker • Thinking • Music • Hot water • Startle

12. Axis 3 – Syndrome diagnosis From a long list ! • Wests (infantile spasms) • Lennox- Gastaut (stare, jerk and fall epilepsy) • Landau-Kleffner (aquired aphasia/ epilepsy syndrome) • Othahara (early infantile epileptic encephalopathy) • Etc. • Conditions with epileptic seizures that do not require a diagnosis of epilepsy - benign neonatal seizures - febrile seizures - single seizures - isolated single cluster

13. Axis 3 – Syndrome diagnosis Or by syndrome groups • Idiopathic focal epilepsies – benign Rolandic - Panayiotopoulos • Familial - AD nocturnal frontal lobe epilepsy - familial temporal lobe epilepsy • Symptomatic focal epilepsies – Rasmussens • Idiopathic generalised – childhood absence ep. • Reflex epilepsies – startle / photosensitive ep. • Epileptic encephalopathies –Othahara/Dravet/ Wests/Lennox Gastaut/Landau Kleffner

14. Axis 3 – Syndrome diagnosis Or keep it simple • Idiopathic / symptomatic / probably symptomatic (replaces ‘cryptogenic’) • Benign vs. malignant

15. Axis 3 - Syndrome diagnosis • Benign – single type of attack - focal or selective - provoked - no cerebral impairment - good outcome - treatment - ?needed ?which drug ?how long

16. Axis 3 - Syndrome diagnosis • Malignant – multiple seizure types - intractable - mental impairment - behaviour problems - drug response disappointing - avoid polytherapy - avoid toxicity

17. Axis 3 - Syndrome diagnosis Advantages and disadvantages • Reasonable indicator of prognosis • Guides management eg drug choice • Difficult to diagnose • Disagreement • Not a guide to pathophysiology

18. Axis 4 - Aetiology • Genetic – juvenile myoclonic 6p/15q/CHRNA7 - AD frontal lobe ep. 20q/CHRNA4 - Wolf Hirschhorn - Rett, Angelman etc. - neurocutaneous syndromes • Developmental brain abnormality - heterotopia, lissencephaly etc. • Aquired structural brain abnormality - brain injury • Metabolic etc.

19. Axis 5 - Impairment • Optional designation of degree of impairment • WHO ICIDH-2 International classification of functioning and disability, 1999

20. Key Questions for any child with funny turns, including those with a diagnosis of epilepsy • Is it epilepsy? • What type of seizure is it? • Is there an epilepsy syndrome? • Is there an underlying aetiology? • What is the correct management?

21. Coping with diagnostic uncertainty • Share it with child and family • Don’t rely on the EEG • Don’t bow to the temptation of a ‘trial of anticonvulsants’ • Use video (camcorder/mobile phone etc) • Time is a great diagnostician – better to delay the diagnosis than wrongly label the child as epileptic and commit them to anticonvulsant treatment • Remember even paediatric neurologists get it wrong!

22. Best books on paediatric epilepsy for the jobbing clinician • Epilepsy in Childhood and Adolescence, Appleton and Gibbs, 2004 publ. Martin Dunitz – only 178 pages! • The Epilepsies ; Seizures, Syndromes and Management, Panayiotopoulos, 2005,publ. Bladon – 540 pages – ask your UB Pharma rep. for a free copy!

23. Case examples • History will be given • 5 key questions –have you remembered them? • Watch video / EEG – has it modified your diagnosis? • Use ILEA 5 axis scheme –have you remembered them? • Management • Discussion