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Difficult epilepsies - medical treatment

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Severe paediatric epilepsy syndromes ... of signs (stiffening, jerking, urinary incontinence) or an abnormal EEG. Misdiagnosis results in ... – PowerPoint PPT presentation

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Title: Difficult epilepsies - medical treatment


1
Difficult epilepsies - medical
treatment
  • Dr Rajat Gupta
  • Consultant Paediatric Neurologist
  • Birmingham Childrens 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
    (Otaharas 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
AED Seizure type
CBZ Absence, myoclonic, GTC
VGB Absence, myoclonic
High dose PHB Absence
LTG in SMEI Myoclonic, GTC
GBP Absence, myoclonic
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

9
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10
Typical absence epilepsy of childhood
  • gt5 of all children with epilepsy
  • 3-12 years
  • male/female 13
  • Prompt onset and cessation of impaired
    consciousness. Often accompanied by eyelid
    flutter or automatisms.
  • Valproate, ethosuximide
  • Remit 75, TC seizures 40, 30
    cognitive impairment

11
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12
Wests 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, Leighs 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
14
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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 (Sandifers 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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