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    Add as FriendFits, faints and funny turns in childhood - Partners in Paediatrics

    by: Rogers

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    1 : Difficult epilepsies - medical treatment Dr Rajat Gupta Consultant Paediatric Neurologist Birmingham Children’s Hospital
    2 : What are difficult epilepsies? Overview of AEDs Some more common epilepsy syndromes – difficult and not so difficult! Common pitfalls INTERACTIVE
    3 : Severe paediatric epilepsy syndromes Those epilepsy syndromes whose natural history is one of ensuing mental retardation usually with accompanying seizures and sometimes accompanying motor abnormality
    4 : Early infantile epileptic encephalopathy (Otahara’s syndrome) Early myoclonic encephalopathy West syndrome and infantile spasms Lennox-Gastaut syndrome Severe myoclonic epilepsy of infancy Myoclonic astatic epilepsy of childhood Other, potentially malignant syndromes
    5 : Medications Standard medications: Sodium valproate for generalised seizures Carbamazepine for partial seizures Ethosuximide for absences Newer medications: Lamotrigine - Topiramate - Levetiracetam Vigabatrin - Oxcarbazepine - Rufinamide Stiripentol - Zonisamide - Gabapentin Tiagabine Older Drugs: Phenytoin - Phenobarbitone - Benzodiazepines Steroids
    6 : UK NICE recommendations for the use of new antiepileptic drugs - 2002 New antiepileptic drugs should be considered within their licensed indications: If established drugs (typically carbamazepine or valproate) have failed. If the most appropriate older drug is contraindicated. If an older drug could interact with other medications (including the oral contraceptive pill). If the older drugs are already known to be poorly tolerated by the individual. If the patient is a woman of childbearing potential (although the safety of newer antiepileptic drugs in pregnancy remains unclear).
    7 : Seizures induced or aggravated by AEDs
    8 : Juvenile myoclonic epilepsy 5-10% of all epilepsy Peak 12-16 years Generalised TC seizures in 90%, juvenile onset absences in 10-30% Sleep deprivation Photosensitivity in 70-80% 60-80% recurrence following AED withdrawal Valproate, lamotrigine
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    10 : Typical absence epilepsy of childhood >5% of all children with epilepsy 3-12 years male/female = 1:3 Prompt onset and cessation of impaired consciousness. Often accompanied by eyelid flutter or automatisms. Valproate, ethosuximide Remit 75%, TC seizures 40%, 30% cognitive impairment
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    12 : West’s syndrome (Infantile spasms, LDs, hypsarrhythmia) 1-5% of all childhood epilepsy 3-12 months 70-80% symptomatic, 20-30% cryptogenic 50% of prenatal origin (malformations, ischaemia, infections, Trisomy 21). TS common cause. Rarely, Leigh’s encephalopathy, pyridoxine dependency, NKH Poor prognosis 70-80% severe developmental delay 50-60% other epilepsies (Lennox-Gastaut synd) Vigabatrin, valproate, lamotrigine, steroids, nitrazepam
    13 : Tuberous sclerosis
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    15 : Lennox-Gastaut syndrome 3% of childhood epilepsy Onset 1-7yrs (peak 3-5yrs) Axial tonic spasms, atonic seizures, atypical absences Slowing and plateauing of cognitive development Cases usually symptomatic 20% progress from West syndrome Polypharmacy common
    16 : Sturge-Weber syndrome variable natural history Early onset resistant partial seizures with hemiplegia and behavioural deterioration may respond to early partial resection or hemispherectomy
    17 : Differential diagnoses of epilepsy 1 Many, especially in childhood and adolescence Misdiagnosis most commonly due to inadequate history taking and incorrect interpretation of signs (stiffening, jerking, urinary incontinence) or an abnormal EEG Misdiagnosis results in inappropriate management, including investigations, treatment and counselling unnecessary stress and psycho-social problems
    18 : Differential diagnoses of epilepsy 2 Jitteriness and benign myoclonus of early infancy Benign paroxysmal torticollis Gastro-oesophageal reflux (Sandifer’s syndrome) Hyperekplexia Tics and ritualistic movements, self-gratification Daydreaming Blue breath-holding attacks, pallid syncopal attacks / reflex anoxic seizures Vasovagal attacks
    19 : Careful and detailed history taking remains the cornerstone of accurate diagnosis in epilepsy Holistic approach to management is important Advice is always available

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