Transcript Funny Turns - Royal Devon & Exeter Hospital

Funny Turns in
Children
Robert Jones, Paediatrician
Andrew Smith, Senior
Neurophysiology Technician
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 videoEEG demonstration
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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?
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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
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ILEA Classification
(Epilepsia,42(6),1-8,2001
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Five axis system-
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Description of ictal semiology
Epileptic seizure type or types
Syndrome diagnosis
Specific aetiology if known
Optional designation of degree of impairment
caused by condition
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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
From the glossary of descriptive
terminology, now standardised
 Differing degrees of detail possible
 Detail needed varies eg. candidate for
surgery, clinical, research
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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
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Axis 2 – Seizure type
Self limiting seizures
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Generalised – tonic-clonic
- clonic
- myoclonic
- typical absence etc
Focal
- sensory
- motor
Gelastic seizures
Hemiclonic seizures
Secondary generalised seizures
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
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Axis 2 – seizure type
Precipitating stimuli for reflex epilepsies
Visual eg flicker
 Thinking
 Music
 Hot water
 Startle
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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
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Axis 3 – Syndrome diagnosis
Or by syndrome groups
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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
Or keep it simple
Idiopathic / symptomatic / probably
symptomatic (replaces ‘cryptogenic’)
 Benign vs. malignant
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Axis 3 - Syndrome diagnosis
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Benign – single type of attack
- focal or selective
- provoked
- no cerebral impairment
- good outcome
- treatment - ?needed
?which drug
?how long
Axis 3 - Syndrome diagnosis
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Malignant – multiple seizure types
- intractable
- mental impairment
- behaviour problems
- drug response disappointing
- avoid polytherapy
- avoid toxicity
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
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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.
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Axis 5 - Impairment
Optional designation of degree of
impairment
 WHO ICIDH-2 International
classification of functioning and
disability, 1999
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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?
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Coping with diagnostic
uncertainty
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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
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!
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Case examples
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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