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 Develop a systematic approach to the child with funny turns Understand the ILEA 2001 Classification Understand the ILEA 2001 Classification Cope with diagnostic uncertainty Cope with diagnostic uncertainty Understand EEG basics Understand EEG basics Learn from case examples with video- EEG demonstration 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? Is it epilepsy? What type of seizure is it? What type of seizure is it? Is there an epilepsy syndrome? Is there an epilepsy syndrome? Is there an underlying aetiology? Is there an underlying aetiology? What is the correct management? 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 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 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 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 Semiology-the study of linguistic signs and symbols

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

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

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

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

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

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

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

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

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

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

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

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

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

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

19. Axis 5 - Impairment Optional designation of degree of impairment Optional designation of degree of impairment WHO ICIDH-2 International classification of functioning and disability, 1999 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? Is it epilepsy? What type of seizure is it? What type of seizure is it? Is there an epilepsy syndrome? Is there an epilepsy syndrome? Is there an underlying aetiology? Is there an underlying aetiology? What is the correct management? What is the correct management?

21. Coping with diagnostic uncertainty Share it with child and family Share it with child and family Don’t rely on the EEG Don’t rely on the EEG Don’t bow to the temptation of a ‘trial of anticonvulsants’ Don’t bow to the temptation of a ‘trial of anticonvulsants’ Use video (camcorder/mobile phone etc) 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 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! Remember even paediatric neurologists get it wrong!

22. Best books on paediatric epilepsy for the jobbing clinician Epilepsy in Childhood and Adolescence, Epilepsy in Childhood and Adolescence, Appleton and Gibbs, 2004 publ. Martin Dunitz – only 178 pages! 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! 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 History will be given 5 key questions –have you remembered them? 5 key questions –have you remembered them? Watch video / EEG – has it modified your diagnosis? Watch video / EEG – has it modified your diagnosis? Use ILEA 5 axis scheme –have you remembered them? Use ILEA 5 axis scheme –have you remembered them? Management Management Discussion Discussion