funny turns in children robert jones, paediatrician andrew smith, senior neurophysiology technician

Funny Turns in Funny Turns in ChildrenChildren

Robert Jones, PaediatricianRobert Jones, Paediatrician

Andrew Smith, Senior Andrew Smith, Senior Neurophysiology Neurophysiology

TechnicianTechnician

Learning ObjectivesLearning Objectives

Develop a systematic approach to Develop a systematic approach to the child with funny turnsthe child with funny turns

Understand the ILEA 2001 Understand the ILEA 2001 ClassificationClassification

Cope with diagnostic uncertaintyCope with diagnostic uncertainty Understand EEG basicsUnderstand EEG basics Learn from case examples with Learn from case examples with

video-EEG demonstrationvideo-EEG demonstration

Key Questions for any child with Key Questions for any child with funny turns, including those with a funny turns, including those with a diagnosis of epilepsydiagnosis 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?

Some definitionsSome definitions Epileptic Seizure-a sudden change in the electrical Epileptic Seizure-a sudden change in the electrical

activity of the brain, accompanied by objective or activity of the brain, accompanied by objective or subjective change in behavioursubjective change in behaviour

Epilepsy-two or more epileptic seizures, or one Epilepsy-two or more epileptic seizures, or one episode of status epilepticusepisode of status epilepticus

Syndrome-a cluster of signs and symptoms, which Syndrome-a cluster of signs and symptoms, which may include evidence from clinical(eg.seizure may include evidence from clinical(eg.seizure type, neurological findings), neurophysiological type, neurological findings), neurophysiological and neuroradiological investigationand neuroradiological investigation

Semiology-the study of linguistic signs and Semiology-the study of linguistic signs and symbolssymbols

ILEA Classification ILEA Classification (Epilepsia,(Epilepsia,4242(6),1-8,2001(6),1-8,2001

Five axis system-Five axis system-

Description of ictal semiologyDescription of ictal semiology Epileptic seizure type or typesEpileptic seizure type or types Syndrome diagnosisSyndrome diagnosis Specific aetiology if knownSpecific aetiology if known Optional designation of degree of Optional designation of degree of

impairment caused by conditionimpairment caused by condition

Key questions and ILEA Key questions and ILEA comparedcompared

QuestionsQuestions Is it epilepsy?Is it epilepsy? Seizure type?Seizure type? Syndrome?Syndrome? Aetiology?Aetiology? Management?Management?

ILEAILEA Semiology(descriptioSemiology(descriptio

n!)n!) Seizure type Seizure type

classifiedclassified Syndrome diagnosisSyndrome diagnosis AetiologyAetiology Impairment (optional)Impairment (optional)

Axis 1 – Ictal Axis 1 – Ictal phenomenologyphenomenology

From the glossary of descriptive From the glossary of descriptive terminology, now standardisedterminology, now standardised

Differing degrees of detail possibleDiffering degrees of detail possible Detail needed varies eg. Detail needed varies eg.

candidate for surgery, clinical, candidate for surgery, clinical, researchresearch

Axis 2 – Seizure typeAxis 2 – Seizure type

From ILEA list of epileptic seizuresFrom ILEA list of epileptic seizures Include localisation within brain if Include localisation within brain if

possiblepossible Include precipitating stimuli for the Include precipitating stimuli for the

reflex epilepsies if possiblereflex epilepsies if possible

Axis 2 – Seizure typeAxis 2 – Seizure typeSelf limiting seizuresSelf limiting seizures Generalised – tonic-clonicGeneralised – tonic-clonic - clonic- clonic - myoclonic- myoclonic - typical absence etc- typical absence etc Focal - sensory Focal - sensory - motor- motor Gelastic seizuresGelastic seizures Hemiclonic seizuresHemiclonic seizures Secondary generalised seizuresSecondary generalised seizures

Axis 2 – Seizure typeAxis 2 – Seizure typeContinuous seizure typesContinuous seizure types Generalised status epilepticusGeneralised status epilepticus - tonic-clonic- tonic-clonic - absence- absence - myoclonic etc.- myoclonic etc. Focal status epilepticusFocal status epilepticus - epilepsia partialis cont.- epilepsia partialis cont. - hemiconvulsive status- hemiconvulsive status - psychomotor status- psychomotor status

Axis 2 – seizure typeAxis 2 – seizure type

Precipitating stimuli for reflex Precipitating stimuli for reflex epilepsiesepilepsies

Visual eg flickerVisual eg flicker ThinkingThinking MusicMusic Hot waterHot water StartleStartle

Axis 3 – Syndrome Axis 3 – Syndrome diagnosisdiagnosis

From a long list !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 Conditions with epileptic seizures that do not require a

diagnosis of epilepsy - benign neonatal seizuresdiagnosis of epilepsy - benign neonatal seizures - febrile seizures- febrile seizures - single seizures- single seizures - isolated single cluster- isolated single cluster

Axis 3 – Syndrome Axis 3 – Syndrome diagnosisdiagnosisOr by syndrome groups Or by syndrome groups Idiopathic focal epilepsies – benign RolandicIdiopathic focal epilepsies – benign Rolandic - Panayiotopoulos- Panayiotopoulos Familial - AD nocturnal frontal lobe epilepsyFamilial - AD nocturnal frontal lobe epilepsy - familial temporal lobe epilepsy- familial temporal lobe epilepsy Symptomatic focal epilepsies – RasmussensSymptomatic 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 KleffnerWests/Lennox Gastaut/Landau Kleffner

Axis 3 – Syndrome Axis 3 – Syndrome diagnosisdiagnosis

Or keep it simple Or keep it simple Idiopathic / symptomatic / probably Idiopathic / symptomatic / probably

symptomatic (replaces symptomatic (replaces ‘cryptogenic’)‘cryptogenic’)

Benign vs. malignantBenign vs. malignant

Axis 3 - Syndrome Axis 3 - Syndrome diagnosisdiagnosis

Benign – single type of attackBenign – 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

Axis 3 - Syndrome Axis 3 - Syndrome diagnosisdiagnosis

Malignant – multiple seizure typesMalignant – 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

Axis 3 - Syndrome Axis 3 - Syndrome diagnosisdiagnosis

Advantages and disadvantagesAdvantages and disadvantages Reasonable indicator of prognosisReasonable indicator of prognosis Guides management eg drug Guides management eg drug

choicechoice Difficult to diagnoseDifficult to diagnose DisagreementDisagreement Not a guide to pathophysiologyNot a guide to pathophysiology

Axis 4 - AetiologyAxis 4 - Aetiology Genetic – juvenile myoclonic 6p/15q/CHRNA7Genetic – 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 abnormalityDevelopmental brain abnormality - heterotopia, lissencephaly etc.- heterotopia, lissencephaly etc. Aquired structural brain abnormalityAquired structural brain abnormality - brain injury- brain injury Metabolic etc.Metabolic etc.

Axis 5 - ImpairmentAxis 5 - Impairment

Optional designation of degree of Optional designation of degree of impairmentimpairment

WHO ICIDH-2 International WHO ICIDH-2 International classification of functioning and classification of functioning and disability, 1999disability, 1999

Key Questions for any child with Key Questions for any child with funny turns, including those with a funny turns, including those with a diagnosis of epilepsydiagnosis 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?

Coping with diagnostic Coping with diagnostic uncertaintyuncertainty

Share it with child and familyShare it with child and family Don’t rely on the EEGDon’t rely on the EEG Don’t bow to the temptation of a ‘trial of Don’t bow to the temptation of a ‘trial of

anticonvulsants’anticonvulsants’ Use video (camcorder/mobile phone etc)Use video (camcorder/mobile phone etc) Time is a great diagnostician – better to delay the Time is a great diagnostician – better to delay the

diagnosis than wrongly label the child as epileptic diagnosis than wrongly label the child as epileptic and commit them to anticonvulsant treatmentand commit them to anticonvulsant treatment

Remember even paediatric neurologists get it Remember even paediatric neurologists get it wrong! wrong!

Best books on paediatric Best books on paediatric epilepsy for the jobbing clinicianepilepsy for the jobbing clinician

Epilepsy in Childhood and Epilepsy in Childhood and Adolescence,Adolescence,

Appleton and Gibbs, 2004 publ. Martin Appleton and Gibbs, 2004 publ. Martin Dunitz – only 178 pages!Dunitz – only 178 pages!

The Epilepsies ; Seizures, Syndromes The Epilepsies ; Seizures, Syndromes and Management, Panayiotopoulos, and Management, Panayiotopoulos, 2005,publ. Bladon – 540 pages – ask 2005,publ. Bladon – 540 pages – ask your UB Pharma rep. for a free copy!your UB Pharma rep. for a free copy!

Case examplesCase examples

History will be givenHistory will be given 5 key questions –have you remembered 5 key questions –have you remembered

them?them? Watch video / EEG – has it modified your Watch video / EEG – has it modified your

diagnosis?diagnosis? Use ILEA 5 axis scheme –have you Use ILEA 5 axis scheme –have you

remembered them?remembered them? ManagementManagement DiscussionDiscussion