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بسم الله الرحمن الرحيم

بسم الله الرحمن الرحيم. بسم الله الرحمن الرحيم. 1. Childhood Epilepsy. Dr. Mohammed AL- Jaradi Sana’a 24-25/4/2013. Helpful tools for diagnosis. Accurate history and description of events Provoking factors Electroencephalogram. 3. Epilepsy syndrome.

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بسم الله الرحمن الرحيم

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  1. بسم الله الرحمنالرحيم بسم الله الرحمن الرحيم 1

  2. Childhood Epilepsy Dr. Mohammed AL- Jaradi Sana’a 24-25/4/2013

  3. Helpful tools for diagnosis • Accurate history and description of events • Provoking factors • Electroencephalogram 3

  4. Epilepsy syndrome A complex of signs and symptoms that define a unique epilepsy condition 4

  5. Etiological categories of syndromes • idiopathic no underlying structural brain disease, presumed genetic • symptomatic seizures are the result of lesional or metabolic disease of the brain • probably symptomatic (cryptogenic) believed symptomatic but no etiology established

  6. Seizures: focal & generalized • Generalized seizure • a seizure whose initial semiology indicates, or is consistent with, more than minimal involvement of both hemispheres • Focal seizure (partial, localization-related) • a seizure whose initial semiology indicates, or is consistent with, initial activation of only part of one cerebral hemisphere 6

  7. Generalized seizure types 7

  8. Generalized epilepsies Idiopathic generalized epilepsies Benign myoclonic epilepsy in infancy Epilepsy with myoclonic-astatic seizures Epilepsy with myoclonic absence seizures Childhood absence epilepsy Juvenile absence, JME, epilepsy with GTC only Generalized epilepsies with febrile seizures plus 8

  9. Epileptic encephalopathy Syndromes of infancyEarly myoclonic encephalopathy; West syndromes Lennox-Gastaut syndrome Epilepsy with continuous spike waves in SW sleep

  10. Focal seizure types 10

  11. Common Childhood Epilepsy Syndromes 11

  12. Infantile spasmsWest syndrome • Onset ages 3-12 months • Brief axial contractions • usually bilateral, may be asymmetrical • typically flexor, may be extensor • usually in clusters, less likely random • typically on awakening, or when drowsy • EEG shows hypsarrhythmia • multifocal spikes • high voltage, chaotic background 12

  13. EEG Record 13

  14. Infantile spasms • Often “cryptogenic” • no definite cause established but child is delayed • Rarely “idiopathic” • no cause established and child is normal • “Symptomatic” cases • congenital infections • CNS malformations • metabolic disorders • genetic syndromes • tuberous sclerosis • perinatal asphyxia • postnatal trauma • acquired infections • immunizations 14

  15. Infantile spasms • Short term treatment to stop spasms, improve EEG • ACTH {Adrenocorticotropic Hormone } effective, dose not established • oral steroids not proven effective • Vigabatrin, especially in tuberous sclerosis • potential retinal toxicity 15

  16. West Syndrome • Infantile Spasms • Hypsarrhythmia on EEG • Developmental regression that begins concurrently with the onset of spasms 16

  17. Outcome • Idiopathic cases: • Normal school 37% • Death 6% • Other seizure types 43% • Neurologic abnormality 31% 17

  18. Outcome • Symptomatic cases: • Normal school - none • Death 37% • Other seizure types 59% • Neurological abnormality 65% • Large number progress to develop Lennox Gastaut Syndrome 18

  19. Rolandic epilepsy • Onset ages 2-12 yrs, peak 5-10 yrs • Characteristic seizures • Infrequent simple partial seizures • tingling in mouth, on face, speech arrest • rare GTCS in sleep • to many drugs 19

  20. Resolve by puberty • Characteristic EEG • high voltage centrotemporal spikes • usually bilateral • Imaging normal • Considered an idiopathic focal epilepsy • some evidence for genetic basis • Treatment • may not be necessary • may respond

  21. Centrotemporal spikes in Benign Rolandic Epilepsy 21

  22. Presentation of “staring spells” • Complex partial seizures • Absence seizures • Behavior staring

  23. Complex Partial Seizures • Commonly temporal lobe focus • Begins in one area, then spreads enough to impair consciousness, but not to evoke a generalized tonic-clonic seizure • Staring is often part of the initial spread • Also can see automatisms…

  24. Automatisms • Coordinated involuntary movements • Consciousness impaired • Patient does not recall activity • Simple • Lip smacking, chewing, uttering sounds, picking, tapping, walking straight or in circles • Complex (behavior involved) • Undressing, chewing inedible objects, wandering, aggression

  25. Treatment of complex partial seizures • Use medications for focal onset seizures • Levetiracetam (Keppra) • Oxcarbamazepine (Trileptal) • Carbamazepine (Tegretol/ Carbatrol) • Lamotrigine (Lamictal) • Topiramate (Topamax) • Zonisimide (Zonegran) • Phenobarbital • Valproic Acid (Depakote) • Lacosamide (Vimpat)

  26. Absence epilepsy

  27. Childhood absence (petit mal) epilepsy • Peak onset age 4-6 years • Many seizures daily • Seizures last seconds • 70+% have associated automatisms • eyelid flutter • simple vocalizations • picking movements • Typical EEG with 3 Hz spike wave • Majority resolve by adolescence

  28. slightly irregular 3 per second spike wave▼

  29. Treating absence seizures • Ethosuximide (Zarontin) • Valproic Acid (Depaken) • Lamotrigine (Lamictal) • Levetiracetam (Keppra)

  30. Absence v. Complex Partial Sz • Absence • < 30 sec • Non-convulsive status epilepticus • Frequent (100’s a day) • Sudden onset, sudden termination • No post-ictal state • Hyperventilation a trigger • EEG: 3-4 Hz spike and wave • Rare interictal abnormalities • Complex Partial • > 1 minute • Rare non-convulsive epilepticus • Occur ≤ daily • Frequent simple, complex automatisms • Evolve to other sz manifestations • Post-ictal state • EEG: Interictal focal abnormalities

  31. Behavioral Staring • Most commonly seen in children with ADD and MR • Occurs when “bored” or over-stimulated • Does not typically make the patient fall or stop an activity abruptly • Can be stopped with close contact / stimulation • Also can be seen in children with epilepsy!

  32. Evaluation of a 1st unprovoked seizure • Good evidence recommends EEG • EEG can help diagnose the event • EEG can identify a specific syndrome • EEG can help with prognosis • timing of EEG not determined Generalized burst Benign focal spike 32

  33. immediate EEG may show abnormality or post ictal slowing • abnormal EEG best predictor of recurrence in neurologically normal children • abnormal neuro exam also strong predictor of recurrence

  34. Evaluation of a 1st unprovoked seizure • Insufficient evidence for routine labs, LP, imaging • consider emergent imaging • if postictal focal deficit, or not at baseline in several hours • consider nonurgent MRI • with significant neuro abnormalities of unknown etiology • a seizure of focal onset • in children under 1 year of age • consider LP • in the very young child (<6 months) • in the patient who fails to return to baseline • in any patient with meningeal signs • if increased ICP suspected, image before LP 34

  35. How likely is a 2nd seizure? • Evidence from multiple Class III studies • Recurrence ranged from 14%-65% • Most recurrences early (in 1st year) • Factors increasing recurrence risk • abnormal EEG • etiology • remote symptomatic seizure recurrence >50% • idiopathic seizure recurrence 30-50% 35

  36. Summary of evidence:Treatment of 1st unprovoked seizure • Most children with a 1st seizure have few or no recurrences • 10% will have many seizures regardless of initial Rx • Rx after 1st vs. 2nd seizure does not affect long term prognosis • Rx in adults and children leads to decreased recurrences 36

  37. Recommendations:Treatment of a 1st Seizure • Anticonvulsant treatment after a 1st seizure must be individualized • treatment is not indicated for prevention of epilepsy • treatment may be considered if risks of recurrent seizure out weigh risks of Rx • Treatment must take into account patient and family preferences 37

  38. Febrile seizures • The most common seizures in childhood • In US affect 2-4% of children < age 5 yrs • 1/3 have at least 1 recurrence • Risk factors for recurrence • first febrile seizure < 1 year of age • low degree of fever at first febrile seizure • family history of febrile seizures • brief duration between fever onset and febrile seizure • 4 risk factors = 70% recurrence • No risk factors = 20% recurrence 38

  39. Febrile seizures • Simple • generalized • and <15 minutes duration • Complex • focal • or >15 minutes • or recurrent within 24 hours 39

  40. Evaluation of the child with simple febrile seizures • Lumbar puncture • if meningeal signs • in infants < 12 months • with prior antibiotic Rx • Blood studies not needed routinely • Imaging not necessary • EEG not necessary in simple febrile seizures 40

  41. Treatment of the child with simple febrile seizures • Effective in reducing recurrences but with potential toxicities • continuous Phenobarbital or valporic acid • intermittent diazepam 41

  42. Thank you! شـــــــــــــــــــــــــــــــــــــكــــــــــــــــــــــــــــــــــــــــرا

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