1. Hints for effective listening 1.Stop talking 2.Be interested 3.Remove distractions 4.Be patient 5.Mind your temper 6.Avoid criticism & arguments 7.Ask questions 8.Paraphrase 9.Stop talking 1

2. PAEDIATRIC SEIZURES & EPILEPTIC SYNDROMES DR. MOHAMMAD AL NASSER Consultant Pediatric Neurologist Department of Pediatrics King Saud University 2

3. OBJECTIVES  Seizures (ZT’s) a symptom NOT a disease  Clinical observation crucial for Dx, classification, and Rx.  R/O other paroxysmal, non-epileptic disorders.  Acute management & prevention of recurrence.  Thoughtful & rational patient work-up  Optimum use of anti-epileptic drugs (AED’s)  Comprehensive patient (not SZ’s) management. 3

4. DEFINITIONS & TERMS  A seizure = abnormal electrical cerebral cortical discharge  clinical alteration (in function and in behavior).  Epilepsy = two or more unprovoked seizures.  Status epilepticus= a seizure lasting more than 30 mins. or repeated seizures with NO regain in consciousness (convulsive or non-convulsive).  Aura, ictus, postical….interictal. 4

5. AETIOLOGY OF SZ’  Primary (idiopathic) -extensive w/u unyielding -genetic vulnerability  Secondary (symptomatic-provoked) -congenital (e.g. anomalies, infections) -acquired (e.g. P-HIE, metabolic…etc.) 5

6. Normal SZ’s threshold Strong provoking factor Low SZ’s threshold No provoking factor Seizure 6

7. International Classification Old Terms General Seizures  AbsencePetit mal - Typical - Atypical  MyoclonicMinor motor  Clonic seizuresGrand mal  Tonic seizuresGrand mal  Tonic-clonic seizures Grand mal  Atonic seizuresAkinetic, drop attacks, minor motor 7

8. International Classification Old terms General Seizures  AbsencePetit mal  MyoclonicMinor motor  Clonic seizuresGrand mal  Tonic seizuresGrand mal  Tonic-clonic seizuresGrand mal  Atonic seizuresAkinetic, drop attacks, minor motor 8

9. International Classification Old terms Partial seizures P. simple seizures (consciousness not impaired) Focal or local seizures  With motor symptomsFocal motor Jacksonian seizures  With somatosensory symptoms Focal sensory  With automatic symptoms  With psychic symptoms 9

10. International Classification Old terms Partial Seizures (cont.) P. Complex seizuresPsychomotor seizures (consciousness impaired)Temporal lobe seizures  Simple partial onset  With impairment consciousness at onset  Partial seizures that secondarily generalize 10

11. APPROACH TO DIAGNOSIS What is the problem? (clinical) Where is the problem? (anatomy) Why is the problem? (pathology) 11

12. DIAGNOSTIC PROCESS Questions to be answered:  Was it a seizure (see DDx of SZ’s)?  Was it provoked (e.g. hunger, T.V., fever…)?  How was the onset (focal generalized)?  Precise description of the event (eye-witness)?  Prior neuro-developmental status?  Findings on neurolofic & G. physical exam…..? 12

13. LABORATORY INVESTIGATIONS  R/O treatable conditions: -CBC, platelets, smear AED’s serum levels -Glucose, Ca, PO 4 -BUN, electrolytes, Cr and CO 2 -Liver function -(+/- CSF & CT scan head) 13

14. LABORATORY INVESTIGATIONS  Neurophysiology: - EEG (regular, sleep-deprived, videotape)  Neuro-imaging: - Ultrasound, SXR, CT scan, MRI (anatomic) PET & SPECT (functional) 14

15. ACUTE MANAGEMENT OF A SEIZURE ATTACK  ABC’s: - suction - O 2 - position  What if: - can not get I.V. access? - SZ is refractory? 15

16. ACUTE MANAGEMENT OF A SEIZURE ATTACK (cont.)  I.V. line: - Get blood - Give anticonvulsant a. glucose, Ca b. benzodiazepine to abort c. long acting AED to prevent recurrence  What aetiologic diagnosis & manage accordingly. 16

17. LONG TERM PROPHYLAXIS  Treat or not to treat?  Choose drug of choice for type of SZ’s.  Single AED & not polypharmacy.  Increase till response or side effects.  Wait 5 x t ½ after each increment.  Add another AED similarly → +/- withdraw 1 st one.  Monitor drug levels (& evidence of side effects) timely & appropriately.  Consider withdrawing AED/s carefully and rationally.  Patient & parent continued education is crucial.  ? Epilepsy surgery? 17

18. QUESTIONS & ISSUES TO BE CLARIFIED  Do seizures damage the brain?  Why there is no cure for epilepsy?  Is patient going to outgrow this?  Can epileptics function normally?  Do AES’s have long-term side effects?  For how long Rx will be continued? 18

19. FEBRILE CONVULSION  Seizure with fever: - Seizure (not shivering [rigors]) - Fever, documented, source outside CNS 19

20. FEBRILE CONVULSION  Simple (typical) FC: -GTC’s -less than 15 mins -no recurrence within 24 hrs. -no postical abnormality 20

21. FEBRILE CONVULSION  Complex (atypical) FC: -Mostly focal -More than 15 mins. -Recur within 24 hrs. 21

22. FEBRILE CONVULSION  Investigations: - Like any seizures disorder -R/O intracranial infection 22

23. FEBRILE CONVULSION Treatment: -Abort the attack -Prophylaxis - No treatment - Daily treatment x 2 yrs. (P.B/VPA) - PRN treatment (Rectal diazepam) 23

24. FEBRILE CONVULSION Treatment:  40% recurrence of FC - young age at onset - family predisposition - complex-type SZ’s - day nursery  10% atypical SZ’s → non-febrile SZ’s (epilepsy) 24

25. INFANTILE SPASMS  Myclonic spasms: - mixed → flexor → extensor  Hypoarrhythmias on EEG  Mental retardation  Typically: -Onset at 3-7/12 of age. -In cluster on awakening -Missed as infantile “colic” 25

26. INFANTILE SPASMS Aetiology: Idiopathic (10-40%): - normal prior development - no brain pathology Symptomatic: - brain malformations; - tuberous sclerosis - others 26

27. INFANTILE SPASMS  Investigations: As other types of SZ’s  Treatment: Steroids, benzodiazepines, valproate, pyridoxine. 27

28. INFANTILE SPASMS  Prognosis: -? Underlying cause -Good in 40% if: - idiopathic - normal development - early treatment -Bad in 60% if: - symptomatic - develop other SZ’s e.g. Lennox-Gastaut. S. 28

29. PAROXYSMAL DISORDERS MIMICKING SZ’s  Decrease cerebral blood flow (CBF)  Sleep disorders.  Movement disorders  Psychologic disorders 29

30. SIMPLE FAINTING (SYNCOPE)  Mostly in school age children.  Usually non-convulsive.  R/O cardiac dysrhythmias.  Precipitant → vasovagal response → venous pooling → decrease CBF.  Rx….. Avoid precipitants 30

31. CYANOTIC BREATH-HOLDING ATTACKS  3% of children  Few months – 4 years  Fright or pain → cry → hold breath in expiration  May show few jerks  Slow EEG intra attack but NOT epileptic  Rx……? 31

32. REFLEX ANOXIC SEIZURES  “Pallid breath-holding” attacks.  Minor trauma → minimal crying → stiff, pale +/- jerks.  Decrease threshold to vagal cardiac inhibitory reflex → a systole.  In 1% of children, mostly 12-18/12 of age.  May co-exist with the cyanotic breath-holding.  ECG should be done.  Rx…..? (transdermal anticholinergic) 32

33. CARDIAC DYSRHYTHMIAS  Consider if: -Syncope: → tonic/clonic movements → prolonged confusion -Exercise-induced “seizures” -Relatives (“epileptic” or sudden deaths)  Prolonged Q-T int. & sick sinus syndromes.  Extensive cardiac investigation is mandatory. 33

34. SLEEP DISORDERS  Nigthmares & night terrors  Narcolepsy & cataplexy  Somnambulish & somniloquy  Sleep apnea  Bruxism, noct, enuresis, noct, myoclonus 34

35. PSYCHOLOGIC DISORDERS  panic attacks  day dreaming  conversion reactions  fictitious epilepsy  hyperventilation syndrome 35

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