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  1. 1. Epilepsy Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  2. 2. EpilepsyDefinition:-Two or more unprovoked seizures (unrelated to fever or an acute cerebral insult) occurring at an interval greater than 24 hrs apart Exceptions –Multiple seizure within 24 hrs Episodes of status epilepticus** F. convulsion & neonatal seizures are excludedIncidence of epilepsy 3%
  3. 3. Aetiology of Epilepsy• Idiopathic 67.6%• Specific etiology - Identifiable in only 30% cases• Congenital……….20% – HIE – Congenital brain anomalies• Infection …………4.0%• Trauma………… 4.7%• Vascular …………1.5%• Neoplastic……….1.5%• Degenerative…….0.7%
  4. 4. ILAE- Epileptic seizures• Partial seizures:1. Simple partial (consciousness retained) – Motor, sensory, Autonomic, psychic2.Complex partial (consciousness impaired) *simple P followed by impaired conscious *Consciousness impaired at onset3. Partial seizures with secondary generalization
  5. 5. ( ILAE): using both clinical data and EEGII. Generalized seizuresA. Absence seizures (petit mal)B. Clonic seizuresC. Tonic seizuresD. Tonic-clonic seizuresE. Atonic seizuresF. Myoclonic seizuresI. Infantile spasmsIII. Unclassified seizures which, due to inadequate data or classification
  6. 6. ILAE epilepsy & Epileptic syndromes 1. Localised - Simple Partial S Complex Partial S Syndromes:- a) Idiopathic age related -Benign childhood focal epilepsy with centrotemporal spikes (rolandic E) b) Symptomatic- chronic progressive E Epilepsia partalis continua
  7. 7. ILAE epilepsy & Epileptic syndromes 2. Generalized – Same Syndromes : A) Idiopathic age related – childhood absence, juvenile absence B) Cryptogenic -West syndrome (infantile spasms) - Lennox-Gastaut Syndrome - Myoclonic seizures
  8. 8. Simple Partial seizures• Motor activity is the most common symptom• Tend to involve the face, neck, extremities• Automatism do not occur, some c/o Aura• Persists -10-20 sec• Remain conscious can verbalize• No postictal phase• EEG-spikes or sharp waves uni or bilaterally or multifocal spikes
  9. 9. Complex partial S• Aura may be +• Brief blank stare or sudden cessation/ pause in activity• Period of altered consciousness may be brief & infrequent• Automatisms are common 50-75%-• In infancy -lip smacking, chewing, swallowing ,excessive salivation (alimentary Automatisms)
  10. 10. Complex partial S• In older children-semipurposeful uncoordinated and unplanned gestures-picking & pulling at clothing or bed sheets, rubbing objects, walking or running in nondirective , repetitive , fearful fashion• Secondary generalization may be +• Duration 1-2 min
  11. 11. Generalized seizures1.Absence S (petit-mal),simple, typical :- -Sudden cessation of motor activity with blank facial expression & flickering of the eyelids - Seizures are subtle - first clue to absence S is often unexplained "daydreaming" and a decline in school performance recognized by a teacher
  12. 12. Absence S (petit-mal),simple, typical - Common age - 4 to 8 yrs or early adolescence, - Prevalent in girls - Incidence 15 to 20% of children - No aura, no postictal state - Duration 30 sec - Countless seizures in a day
  13. 13. Generalized seizuresAbsence S (petit- mal),simple, typical…….. - Body tone normal but head may fall slightly forward -Automatism frequently present -EEG-3/sec spike and generalized wave discharge
  14. 14. Atypical Absence Seizures• Longer duration• Seizure is accompanied by more obvious motor signs consisting of myoclonic movements of face, fingers and extremities, focal or lateralizing features• Mental retardation may be present• Less responsive to anticonvulsants• The EEG - shows a generalized, slow spike-and-wave pattern with a frequency of =2- 2.5cycles/sec
  15. 15. 2.Generalized tonic – clonic grand –mal) Aura – Due to partial onset of seizure , lasts sec –min eg olfactory hallucination, epigastric discomfort, deja-vu, jerking of one limb Tonic phase -rapid discharging of motor cortex cells cause tonic contraction of muscles throughout the body - skeletal muscle undergoes sustained spasm
  16. 16. Tonic phase….• Skeletal muscle undergoes sustained spasm - Laryngeal muscles ,muscles of expiration - forces air out of the lungs through a partially closed glottis - produce "ictal cry." (shrill cry) - Respirations are impaired, secretions pool in the oropharynx, and cyanosis develops - Muscle rigidity: more in antigravity muscles uprolling of eyes or deviated to sides
  17. 17. Tonic phase….• Contraction of the jaw muscles - biting of the tongue.• Lasts 10-30 sec
  18. 18. Generalized tonic – clonic grand –mal) Clonic phase- 1-5 min - Gradually slowing discharge of cortical cells, rhythmic clonic contractions alternating with relaxation of muscle groups - Marked enhancement of sympathetic tone - increases in heart rate, blood pressure, pupil dilated, Loss of sphincter control
  19. 19. Postictal phase• Characterized by :- Semicomatose- Unresponsiveness - deep sleep for 30min to 2hrs • Muscular flaccidity, loss of corneal reflex • Extensor plantar responses • Loss of sphincter control -incontinence , tongue biting • Lasts 30 min – 2 hrs
  20. 20. Generalized tonic – clonic grand –mal) Post-ictal – 30 min – 2 hrs Deep unconsciousness, flaccid limbs, loss of corneal reflexes, extensor plantar responses, Excessive salivation cause stridorous breathing and partial airway obstruction - Patients gradually regain consciousness • Bi-frontal headache, confusion, aching muscles ,sometimes automatic behavior, occasional violence (2- 21/2hours)
  21. 21. Postictal confusion• Bifrontal headache• Confusion• Aching muscles• Sometimes automatic behavior• Occasinal violence• Lasts - 2-2 ½ hours
  22. 22. GTCS - Precipitated by- Infections - Excessive fatique - Emotional stress- Various drugs -theophylline, anti- psychotropic drugs, cipro, benzodiazepines, amphetamine, cocaine, alcohol, flumazenil - Forgetting to take anticonvulsants
  23. 23. The EEGDuring the tonic phase - shows a - progressive increase in generalized low-voltage fast activity, followed by generalized high-amplitude, polyspike dischargesDuring clonic phase - the high-amplitude activity is typically interrupted by slow waves to create a spike-and-wave patternThe postictal EEG - shows diffuse slowing that gradually recovers as the patient awakens
  24. 24. 3.Myoclonic epilepsies of childhood • Repetitive seizures with brief ,symmetric muscular contractions with loss of body tone and falling or slumping forward • Subgroups: A. Benign myoclonus of infancy -confined to neck, trunk, extremities - EEG normal - Good prognosis - improves by age 2yrs - No medications needed
  25. 25. B. Typical myoclonic epilepsy of early childhood -Normal birth history, normal milestones -starts at 6 mo -4 yrs peak age-2 ½ yrs -Family history + in 1/3rd cases -EEG- fast spike waves -50% seizure free few yrs after treatment -Learning language problem, emotional, behavioral disorder - MR may occur in some
  26. 26. C. Complex myoclonic epilepsies • Begin-1st yr of life, • Starts as generalized seizure associated with URTI • Presents as status epilepticus-poor prognosis • 1/3rd have delayed development, • H/o birth asphyxia ,microcephaly • No family h/o epilepsy • EEG- interictal slow spike waves • Refractory to anticonvulsants
  27. 27. D. Juvenile myoclonic epilepsy (janz syndrome) • Begin at age 12-16 yrs • Initially hair combing, tooth brushing become difficult • Few yrs later with myoclonus have GTC seizure • EEG –abnormal • Responds to valproate required lifelong
  28. 28. E. Progressive myoclonic epilepsies • Neurologic abnormalities-cerebellar & extrapyramidal signs ++ • Mental deterioration is characteristic feature • Genetic disorder –grave prognosis • EEG-abnormal • Valproic acid + clonazepam is effective
  29. 29. 4.Infantile spasms• 4-8 mo of life• Brief symmetrical contractions of neck, trunk, extremities-sudden dropping of head & flexion of arms - ‘Salaam fit’• EEG-Hypsarrhythmic pattern – diffuse high voltage slow spike and chaotic activity
  30. 30. 4.Infantile spasms• Types –• Flexor spasms• Extensor spasms-least common• Mixed infantile spasms-most common• More tendency when drowsy or immediately on awaking
  31. 31. 4.Infantile spasmsTwo groups1. Crytogenic -10-20% cases - Normal birth history - Normal development - Neurologic exam, CT,MRI head- normal - No risk factors
  32. 32. 4.Infantile spasms2. Symtomatic- - 80-90% Related to several factors:- Antenatal-HIE, cong infections, inborn errors of metabolism, neurocutaneous syndromes, Cong anomalies of brain Post natal–CNS infections, head Trauma,HIE -80-90% -MR
  33. 33. Lennox –Gastaut Syndrome• Onset –late infancy or childhood• Mixed seizure- myoclonic, atypical absence ,generalized tonic-clonic, partial• Prognosis unsatisfactory• Triad-1.Atypical absences, axial tonic seizures, drop attacks2. Typical EEG pattern - Slow spike and waves on awake EEG & 10Hz bursts during sleep3.Psychomotor retardation
  34. 34. Management• 1st seizure: Blood sugar, electrolytes,calcium, EEG, CT,MRI,CSF-when indicated** Normal-Negative family history-No treatment ,only close observation**Abnormal-treat underlying cause/AED if necessary**Normal except EEG abnormal-Idiopathic Epilepsy-treatment
  35. 35. Did the child had seizure ? NO YES SEIZURE MIMICS INITIAL RECURENT SEIZURE SIEZURE Drug compliance RBS , electrolyte, Improper drug EEG?, CT?, CSF? Metabolic icSol Degenerative CNS Drug interactionABNORMAL NORMAL NORMALSYMPTOMATIC SIEZURE EXCEPT EEG Isolated 1st sizure IDIOPATHICTREAT underlying cause No family history EPILEPSYAED if necessary No TREATMENT Close observation
  36. 36. NORMAL EXCEPT EEG IDIOPATHIC EPILEPSY Classify seizure typeGood control Poor controlRegular follow up HospitalizeAED drug levels EEG , video monitoringMonitor toxicity Readjust drugsEEG CT, MRI, FREQUENT FOLLOW UPS
  37. 37. When To Initiate AED Therapy(1) An abnormal neurologic examination(2) Seizures presenting as status epilepticus(3) Postictal Todds paralysis(4) A strong family history of seizures(5) An abnormal EEG• Antiepileptic drug therapy should be started in any patient with recurrent seizures of unknown etiology
  38. 38. Selection Of AED• Carbamazepine, phenytoin, or lamotrigine is currently the initial drug of choice for partial seizures, including those that secondarily generalize• Valproic acid is currently - best initial choice for primarily generalized, tonic-clonic seizures.• Lamotrigine, followed by carbamazepine and phenytoin, are suitable alternatives.
  39. 39. • Valproic acid is effective in - absence, myoclonic, and atonic seizures - drug of choice for generalized epilepsy syndromes having mixed seizure types
  40. 40. Treatment1.Establish diagnosis2. Decide about need for AED3. Choice of most suitable drug-age, type of seizure, economic condition of patient4.Proper F/U**Go for monotherapy**start with small dose & gradually increase to maintenance dose over few weeks
  41. 41. AED Choice Based on S. TypeSeizure First choice OtherstypePartial S CBZ Phenytoin, Valproic acid, Phenobarbital,Tonic- Valproic acid Topiramate, Phenobarbital,clonic Lamotrigine, Zonisamide, Felbamate,phenytoin ,carbamazepineAtonic Valproic acid Topiramate, Lamotrigine, Zonisamide,Felbamate
  42. 42. AED Choice Based on S. TypeSeizure First choice OtherstypeChildhood Ethosuximide Lamotrigine,valproic acid,Absence Zonisamide, clonazepamepilepsy felbamateJuvenile Valproic acid Lamotrigine, topiramate, zonisamideAbsenceEpilepsyInfantile ACTH, Topiramate, Zonisamidespasm Vigabatrin ,prednisolone,valproic acid
  43. 43. AED Choice Based on S. TypeSeizure First choice OtherstypePartial S CBZ Phenytoin, Valproic acid, Phenobarbital,Tonic- Valproic acid Topiramate, Phenobarbital,clonic Lamotrigine, Zonisamide, Felbamate,phenytoin ,carbamazepineAtonic Valproic acid Topiramate, Lamotrigine, Zonisamide,Felbamate
  44. 44. AED Choice Based on Epilepsy TypeEpilepsy First-choice OtherstypeJuvenile Valproic acid Lamotrigine, topiramate,Myoclonic zonisamide, felbamateEpilepsy ,phenobarbitone,clonazepamBenign Rolandic Carbamazepine Gabapentine,topiramate,lamoEpilepsy trigine,valproic acid,phenytoinLennox- Valproic acid Topiramate,lamotrigine,Gastaut zonisamide, felbamate,clonazepam
  45. 45. Adjunctive T when S poorly controlled • Gabapentine, clobazam, lamotrigine, to piramate, vigabatrin • Surgical Rx-Resection of corpus callosum, focal resection of parts of cerebral cortex involved as epileptic foci
  46. 46. WHEN TO DISCONTINUE THERAPYDuration: should be invidualized as there is no unanimity in literature(1) Complete medical control of seizures for 1 to 5 years(2) In children seizures that are easily controlled and have no underlying structural lesions, no MR, no neurological deficits need 2-3 yrs seizure free course(3) Normal EEG
  47. 47. Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]