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Difficult epilepsies - medical treatment. Dr Rajat Gupta Consultant Paediatric Neurologist Birmingham Children’s Hospital. What are difficult epilepsies? Overview of AEDs Some more common epilepsy syndromes – difficult and not so difficult! Common pitfalls INTERACTIVE.
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Difficult epilepsies - medical treatment Dr Rajat Gupta Consultant Paediatric Neurologist Birmingham Children’s Hospital
What are difficult epilepsies? • Overview of AEDs • Some more common epilepsy syndromes – difficult and not so difficult! • Common pitfalls • INTERACTIVE
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
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
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
UK NICE recommendations for the use of new antiepilepticdrugs - 2002 New antiepileptic drugs should beconsidered within their licensed indications: • If establisheddrugs (typically carbamazepine or valproate) have failed. • Ifthe most appropriate older drug is contraindicated. • If an olderdrug could interact with other medications (including the oralcontraceptive pill). • If the older drugs are already known tobe poorly tolerated by the individual. • If the patient is awoman of childbearing potential (although the safety of newerantiepileptic drugs in pregnancy remains unclear).
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
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
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
Tuberous sclerosis
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
Sturge-Weber syndrome • variable natural history • Early onset resistant partial seizures with hemiplegia and behavioural deterioration may respond to early partial resection or hemispherectomy
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
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 Differential diagnoses of epilepsy 2
Careful and detailed history taking remains • the cornerstone of accurate diagnosis in epilepsy • Holistic approach to management is important • Advice is always available