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
3Severe 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
5Medications
- 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
6UK 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).
7Seizures 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
8Juvenile 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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10Typical 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
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12Wests 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
13Tuberous sclerosis
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15Lennox-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
16Sturge-Weber syndrome
- variable natural history
- Early onset resistant partial seizures with
hemiplegia and behavioural deterioration may
respond to early partial resection or
hemispherectomy
17Differential 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
18Differential 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