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Funny Turns in Children Robert Jones, Paediatrician

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    Funny Turns in Children Robert Jones, Paediatrician Funny Turns in Children Robert Jones, Paediatrician Presentation Transcript

    • 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 video-EEG demonstration
    • 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
      • 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
      • 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)
    • 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
      • 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
      • 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
      • 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
      • Precipitating stimuli for reflex epilepsies
      • Visual eg flicker
      • Thinking
      • Music
      • Hot water
      • Startle
    • 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
      • 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
      • Or keep it simple
      • Idiopathic / symptomatic / probably symptomatic (replaces ‘cryptogenic’)
      • Benign vs. malignant
    • 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
      • 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
    • 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
      • 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
      • 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
      • 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!
    • 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