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    Add as FriendFunny Turns in Children

    by: Rogers

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    1 : Funny Turns in Children Robert Jones, Paediatrician Andrew Smith, Senior Neurophysiology Technician
    2 : 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
    3 : 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?
    4 : 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
    5 : 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
    6 : 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)
    7 : 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
    8 : 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
    9 : 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
    10 : 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
    11 : Axis 2 – seizure type Precipitating stimuli for reflex epilepsies Visual eg flicker Thinking Music Hot water Startle
    12 : 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
    13 : 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
    14 : Axis 3 – Syndrome diagnosis Or keep it simple Idiopathic / symptomatic / probably symptomatic (replaces ‘cryptogenic’) Benign vs. malignant
    15 : Axis 3 - Syndrome diagnosis Benign – single type of attack - focal or selective - provoked - no cerebral impairment - good outcome - treatment - ?needed ?which drug ?how long
    16 : Axis 3 - Syndrome diagnosis Malignant – multiple seizure types - intractable - mental impairment - behaviour problems - drug response disappointing - avoid polytherapy - avoid toxicity
    17 : 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
    18 : 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.
    19 : Axis 5 - Impairment Optional designation of degree of impairment WHO ICIDH-2 International classification of functioning and disability, 1999
    20 : 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?
    21 : 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!
    22 : 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!
    23 : 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

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