Funny Turns

Funny Turns in Children: 

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

Learning Objectives: 

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

Key Questions for any child with funny turns, including those with a diagnosis of epilepsy: 

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?

Some definitions: 

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

ILEA Classification (Epilepsia,42(6),1-8,2001: 

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

Key questions and ILEA compared: 

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

Axis 1 – Ictal phenomenology: 

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

Axis 2 – Seizure type: 

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

Axis 2 – Seizure type: 

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

Axis 2 – Seizure type: 

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

Axis 2 – seizure type: 

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

Axis 3 – Syndrome diagnosis: 

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

Axis 3 – Syndrome diagnosis: 

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

Axis 3 – Syndrome diagnosis: 

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

Axis 3 - Syndrome diagnosis: 

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

Axis 3 - Syndrome diagnosis: 

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

Axis 3 - Syndrome diagnosis: 

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

Axis 4 - Aetiology: 

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.

Axis 5 - Impairment: 

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

Key Questions for any child with funny turns, including those with a diagnosis of epilepsy: 

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?

Coping with diagnostic uncertainty: 

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!

Best books on paediatric epilepsy for the jobbing clinician: 

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!

Case examples: 

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