LMCC Review: Pediatric Neurology

1. LMCC Review:Pediatric Neurology Asif Doja, MEd, MD, FRCP(C) March 27th, 2012

2. Outline • Seizures • Febrile Seizures • Status Epilepticus • Headache

3. Seizures

4. Question 1 Someone can be diagnosed with epilepsy if they have: A. More than one febrile seizure B. More than one afebrile seizure C. Seizures in the context of hypoglycemia D. One seizure and a history of brain injury

5. Question 2 All of the following seizure types are classified as “generalized” seizures EXCEPT: A. Complex partial seizures B. Absence seizures C. Tonic-clonic seizures D. Atonic seizures

6. Question 3 All of the following are features of Absence seizures EXCEPT: A. Lack of an aura or warning B. Impairment in consciousness C. Post-ictal drowsiness/lethargy D. 3 Hz spike and wave on EEG

7. Question 4 Which of the following is an appropriate first line treatment for an 8 year old child with epilepsy? A. Bromide therapy B. Ketogenic Diet C. Carbemazepine D. Phenobarbital

8. Question 5 A 9 year old child presents with recurrent episodes of waking in the morning with facial twitching, dysarthria and normal level of consciousness. The most likely diagnosis is: A. Transient Ischemic Attacks B. Benign Epilepsy of Childhood with Rolandic Spikes C. Juvenile Myoclonic Epilepsy D. Facial tics

9. Definitions • Seizure: Paroxysmal discharge of neurons resulting in behaviour change, motor or sensory dysfunction • Epilepsy: > 1 unprovoked seizure

10. Was it a Seizure? • Differential Diagnosis • Syncope • Breath Holding • Night Terrors • Tics • GERD • etc

11. Syncope vs Seizure • Vasovagal reflex • Usually happens when standing up • Lightheaded feeling • Pale, cold, clammy • Loss of consciousness and fall • Tremble but no tonic-clonic movements • No post-ictal lethargy

12. Focal Simple Partial Complex Partial Partial Seizure with 2O Generalization Generalized Generalized Tonic-Clonic Tonic Clonic Absence Atonic Myoclonic Focal vs. Generalized Seizures

13. Complex Partial Aura ~ 30 sec or more Decr LOC Automatisms Post-ictal period EEG: focal epileptiform abnormality Hyperventialtion has no effect Absence No aura Lasts few seconds Decr LOC May have automatisms No post-ictal period EEG: 3 HZ spike and wave Provoked by hyperventialtion How to differentiate “Staring Spells”

14. Investigations and Treatment • Neuroimaging if focal findings present • May do EEG after first seizure • Treatment if patient has 2 or more seizures • Commonly used: Carbemazepine, Valproic Acid, Phenobarbital • Many other newer anticonvulsants ie Topiramate, Levotiracetam • (For refractory patients: Ketogenic Diet, Epilepsy surgery)

15. West Syndrome Infantile Spasms Onset in 1st year Symmetrical contractions of trunk/extremities EEG: hypsarrythmia Poor prognosis Lennox Gastault Onset age 3-5 Multiple seizure types Developmental delay EEG: slow spike and wave Many have history of infantile spasms Epilepsy Syndromes

16. Benign Epilepsy of Childhood with Rolandic Spikes (BECRS) 5-10 years Simple partial seizures involving face Remits spontaneously, no treatment Juvenile Myoclonic Epilepsy 12-16 years Myoclonus and GTC seizures Good prognosis, but requires lifelong treatment with Valproic Acid Epilepsy Syndromes

17. Question 1 Someone can be diagnosed with epilepsy if they have: A. More than one febrile seizure B. More than one afebrile seizure C. Seizures in the context of hypoglycemia D. One seizure and a history of brain injury

18. Question 2 All of the following seizure types are classified as “generalized” seizures EXCEPT: A. Complex partial seizures B. Absence seizures C. Tonic-clonic seizures D. Atonic seizures

19. Question 3 All of the following are features of Absence seizures EXCEPT: A. Lack of an aura or warning B. Impairment in consciousness C. Post-ictal drowsiness/lethargy D. 3 Hz spike and wave on EEG

20. Question 4 Which of the following is an appropriate first line treatment for an 8 year old child with epilepsy? A. Bromide therapy B. Ketogenic Diet C. Carbemazepine D. Phenobarbital

21. Question 5 A 9 year old child presents with recurrent episodes of waking in the morning with facial twitching, dysarthria and normal level of consciousness. The most likely diagnosis is: A. Transient Ischemic Attacks B. Benign Epilepsy of Childhood with Rolandic Spikes C. Juvenile Myoclonic Epilepsy D. Facial tics

22. Febrile Seizures

23. Question 1 Which of the following is NOT a feature of a typical febrile seizure? A. Onset between ages 6 months – 6 years B. Duration of < 15 minutes C. Only one seizure in 24 hour span D. Patients usually have pre-existing developmental delay

24. Question 2 Which of the following is FALSE regarding atypical febrile seizures? A. They may show clonic jerking on only one side of the body B. The patient is at no increased risk for further febrile seizures. C. The patient can present in status epilepticus D. The patient can show focal abnormalities on neurologic exam.

25. Question 3 A 8 month old female has one typical febrile seizure, then 2 months later has another. With respect to anticonvulsants, you would prescribe: A. Phenobarbital B. Carbemazepine C. Valproic Acid D. None, as the patient does not require treatment

26. Question 4 A 7 month old male has a typical febrile seizure. With respect to doing a lumbar puncture, the AAP guidelines state that you should: • Not do an LP • Do an LP if the temperature is > 39 degrees C. Do an LP only if there are meningeal signs D. Do an LP irregardless of the physical exam findings

27. Question 5 What is the risk of developing epilepsy in a child with a typical febrile seizure? A. 1%, the same as the general population B. 2-3% C. 10-15% D. 33%

28. Febrile Seizures • 3-5% of all children • Ages 6 months to 6 years • Usually GTC

29. Typical Duration < 15 min No focality Does not recur in 24-hour period No hx of developmental delay Atypical Duration > 15 min Focal findings during seizure or after exam > 1 in 24 hours Previous History of Developmental Delay Typical vs Atypical Febrile Seizures

30. Risk of Recurrence • 33% chance of recurrence (75% occur within 1 year) • Risk Factors: • Family history of feb. con. or epilepsy • Short duration of fever prior to seizure • Developmental / Neurological problems • Atypical febrile seizure

31. Investigations • History and Physical – determine source of fever • EEG and Neuroimaging only needed in atypical cases • LP: • If < 12 months: Do LP • If 12-18 months: Consider LP • If > 18 months: Only if meningeal signs present

32. Management • Reassurance • Risk of developing epilepsy is 2-3% (1% in general population) • Antipyretics and fluids for comfort (neither prevent seizures) • No need for anticonvulsants

33. Question 1 Which of the following is NOT a feature of a typical febrile seizure? A. Onset between ages 6 months – 6 years B. Duration of < 15 minutes C. Only one seizure in 24 hour span D. Patients usually have pre-existing developmental delay

34. Question 2 Which of the following is FALSE regarding atypical febrile seizures? A. They may show clonic jerking on only one side of the body B. The patient is at no increased risk for further febrile seizures. C. The patient can present in status epilepticus D. The patient can show focal abnormalities on neurologic exam.

35. Question 3 • A 8 month old female has one typical febrile seizure, then 2 months later has another. With respect to anticonvulsants, you would prescribe: • A. Phenobarbital • B. Carbemazepine • C. Valproic Acid • D. None, as the patient does not require treatment

36. Question 4 A 7 month old male has a typical febrile seizure. With respect to doing a lumbar puncture, the AAP guidelines state that you should: A. Not do an LP B. Do an LP if the temperature is > 39 degrees C. Do an LP only if there are meningeal signs D. Do an LP irregardless of the physical exam findings

37. Question 5 What is the risk of developing epilepsy in a child with a typical febrile seizure? A. 1%, the same as the general population B. 2-3% C. 10-15% D. 33%

38. Status Epilepticus

39. Question 1 Status Epilepticus is defined as: A. 30 minutes or > of continuous seizure activity B. Recurrent seizures with no intervening normal level of consciousness for > 30 min C. A and B D. None of the above

40. Question 2 A 5 year old boy presents to the ER with a 45 minute GTC seizure. What is your initial management? A. ABC’s B. Stat CT head C. Lorazepam 0.1mg IV push D. Tox screen

41. Question 3 Which of the following metabolic disturbances is MOST likely to cause seizures? A. High Potassium B. High Chloride C. Low urea D. Low glucose

42. Question 4 First line anticonvulsant treatment in status epilepticus should be: A. Lorazepam B. Phenytoin C. Phenobarbital D. Thiopentol coma

43. Status Epilepticus • 30 minutes or > of continuous seizure activity • Recurrent seizures with no intervening normal level of consciousness for > 30 min

44. Status Epilepticus • ABC’s • Oxygen / pulse oximetry • Bag-valve support or intubation if req’d • IV access • Check blood sugar -- give dextrose if low (2-4 ml/kg of 25% solution)

45. Status Epilepticus • Anticonvulsants: • Benzodiazepines ie Lorazepam (0.1 mg/kg IV), can repeat X1 • If fails, Phenytoin 20mg/kg (no faster than 1 mg/min) • If fails, Phenobarbital 20 mg/kg (no faster than 1 mg/min) • If fails, will need to go to ICU for barbituate coma (ie thipentol) or midazolam infusion

46. Question 1 Status Epilepticus is defined as: A. 30 minutes or > of continuous seizure activity B. Recurrent seizures with no intervening normal level of consciousness for > 30 min C. A and B D. None of the above

47. Question 2 A 5 year old boy presents to the ER with a 45 minute GTC seizure. What is your initial management? A. ABC’s B. Stat CT head C. Lorazepam 0.1mg IV push D. Tox screen

48. Question 3 Which of the following metabolic disturbances is MOST likely to cause seizures? A. High Potassium B. High Chloride C. Low urea D. Low glucose

49. Question 4 First line anticonvulsant treatment in status epilepticus should be: A. Lorazepam B. Phenytoin C. phenobarbital D. Thiopentol coma

50. Headache