Epilepsy

1. Epilepsy Xuqin Chen Lecturer of Pediatric Neurology Department Children�s Hospital of Soochow University

2. Epilepsy (p130) Aim and claim 1. Know the features of common types of childhood seizures 2. be able to give advice to the parents of a child with febrile seizures 3. know the steps to manage children with epilepsy and status epilepticus

3. Main contents 1. Introduction of epileptic seizures 2. the classification of epileptic seizures and their clinical features 3.the diagnosis of epilepsy and epileptic seizures 4.the management of epilepsy

4. What is� Epilepsy Epileptic seizure Epilepsy syndrome

5. Epilepsy Chronic brain disorder of various etiologies characterized by recurrent unprovoked seizures

6. Epileptic Seizure Discrete epileptic events due to transient, hypersynchronous, abnormal neuronal behavior.

7. Epilepsy syndrome Grouping of similar epileptic patterns according to seizure type, EEG, age of onset, familial episodes, prognosis, other clinical signs.

8. Epilepsy: a chronic disorder Epileptic seizure: a event Epilepsy syndrome: grouping of epileptic patterns

9. Prevalence of active epilepsy: 0.4-1% Life time cumulative incidence:1.4-3.3%

10. types of epileptic seizures

11. Classification of epilepsies and epilepsy syndromes

12. Causes of epilepsy About 75%no cause ---idiopathic� genetic 25%---symptomatic

13. Causes of symptomatic epilepsy

14. Diagnosis: History: detail description of the event (seizure type, time span, accompany manifestation, and so on.) Investigations: electroencephalogram (EEG ) evidence

15. EEG--- routine EEG�40% positive in patient ambulatory EEG monitoring--- raise the positive rate �80% Video EEG--- especially suitable for those frequent episodes

16. Neuroimaging: Indications: Partial seizure Intractable,difficult control seizure Focal neurological deficit Neurocutaneous syndrome or neurodegenerative disorders

17. Topic2. Epilepsy(cont.8) Methods: CT scan MRI

18. Topic 3. Epilepsy(cont.9) Other investigations Plasma and urine amino acids Biopsy of skin or muscle WBC enzymes DNA analysis

19. Epilepsy diagnosis established A single anticonvulsant is started . Dose built up until fit control without drug-related side effect. Monotherapy failed, combination. Serum anticonvulsant level monitored needed especially with valproate, phenytoin, phenobarbitone. Cessation of medication be considered after a 1-2-year fit-free period.

20. The drug of choice is depend on the seizure type sodium valproate---generalized epilepsy carbamazepine-----partial epilepsy

21. Management Special activities swimming: supervised bathing: supervised cycling: wear helmet climbing: avoided

22. Some Conditions that may be mistaken for Seizures Syncope Non-epileptic seizures (pseudoseizures) Breath holding spells (children) Paroxysmal REM sleep behavior (benign myoclonus, night terrors, etc.) Panic attack

23. Management for the seizure like disorders In-hospital observation Repeated EEGs Video-EEG, preferably

24. Epilepsy (cont�) Some important epilepsy syndromes

25. Some important epilepsy syndromes

26. West Syndrome Definition: A triad of Infantile spasms( repetitive symmetrical contractions of neck, trunk, limbs) chaotic (Hypsarrhythmia) Developmental delay (CP, cognitive disability, mental retardation, etc.)

27. West Syndrome Incidence: 2.9~4.5/100,000 live births( boys are affected in 60% of all the cases) Age of onset: 95%<12 months 70% age 2-8 months Peak age 4-6 months

28. Infantile Spasms Flexor, extensor and mixed Trunk and extremities Mainly in clusters (1-200, avg 15-20) Mainly upon arousal and before sleep. At times asymmetric.

29. Etiology (n=53)

30. Treatment ACTH Vigabatrin Nitrazepam, clonazepam Valproate Pyridoxine (Vitamin B6) Others: felbamate, gammaglob., Topiramate,

31. Lennox-Gastaut Syndrome Median age of onset : 1-2 y (may be later) No underlying cause in 30-50% cases. Slow bilateral synchronous 2 hz spike and wave on EEG Most children are or become mentally handicapped Seizures may be tonic, atonic, myoclonic, clonic or atypical absence in type. Often mixed types

32. Lennox-Gastaut Syndrome Treatment usually not very effective (refractory,10% have a reasonable outcome) Valproate and benzodiazepines used poor prognosis, global development delay (motor, cognitive, language).

33. Childhood absence epilepsy: 4~12 year age , usually affect girls. Etiology: idiopathic Thrigger: hyperventilation

34. Childhood absence epilepsy sudden loss of consciousness, staring, eyelid flickering less than 30 seconds, EEG change---spike wave discharge(3Hzs) sodium valproate affective Good prognosis

35. Febrile seizures Definition of FS - NIH Consensus Conference :an event in infancy or childhood, usually occurring between 3 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause.

36. FC - Age of Onset By definition: 3 months - 6years 90% : 6 months - 3 years 4% : < 6 months 6% : > 3 years About half in the 2nd year - mostly during the 2nd half.

37. Complex FC Definitions: Prolonged ( > 15 min.) Partial seizures Multiple Occurrence: 10-38% of children with FC

38. Subcommittee: Neurodiagnostic Evaluation of the Child with a first Simple FC LP should be �strongly considered� in infants younger than 12 months. EEG should not be performed. WBC, electrolytes and blood glucose should not be performed. Neuroimaging should not be performed.

39. Recurrence of FC 1/3 will have a 2nd attack 1/2 of those will have a 3rd attack 9% will have more than 3 attacks

40. FC - Subsequent Epilepsy Of children with SFC - 1-1.5% will develop epilepsy at F/U. Of children with CFC - 4-15% will develop epilepsy at F/U. Additional risk factors: Early onset Family epilepsy history

41. FC - Subsequent Epilepsy (cont�) Of the children with complex FC: Partial FC has higher risk for epilepsy Combination of Partial FC & other parameter of complex FC increases the risk.

42. FC - Options for Drug Treatment Phenobarbital: sedation, hyperactivity, irritability - 1/3. Cognitive decline ? Valproic acid: Fatal hepatotoxicity Intermittent diazepam No place for routine anticonvulsant therapy for S FC

43. Status Epilepticus  Seizures continuing or recurring without full recovery for 30 minutes or longer.

45. SE result in Cerebral hypoglycaemia, Lactic acidosis Hypoxia mortality 5% Active resuscitation needed

46. Mortality of Status Epilepticus 2-3% from the SE itself. 25-30% overall (most from the underlying illness). 3% in children. 26% in adults. DeLorenzo, et al., Epilepsia, 1998

47. Emergency treatments for SE Airway protection Metabolic derangements correction Anticonvulsants, General anaesthesia with thiopentone (????)

48. Management Special activities swimming: supervised bathing: supervised cycling: wear helmet climbing: avoided

49. Use of the anti-epilepsy drugs: sodium valproate---generalized epilepsy carbamazepine-----partial epilepsy phenobabitone---- Topic 2. Epilepsy(cont.15)

50. Thank you