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
(No Transcript)
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
(No Transcript)
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
(No Transcript)
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