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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

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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
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
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 type9
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 type10
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 type11
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 diagnosis13
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 diagnosis14
Axis 3 – Syndrome diagnosis

Or keep it simple

  • Idiopathic / symptomatic / probably symptomatic (replaces ‘cryptogenic’)
  • Benign vs. malignant
axis 3 syndrome diagnosis15
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 diagnosis16
Axis 3 - Syndrome diagnosis
  • Malignant – multiple seizure types

- intractable

- mental impairment

- behaviour problems

- drug response disappointing

- avoid polytherapy

- avoid toxicity

axis 3 syndrome diagnosis17
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 epilepsy20
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