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

3. Outline Seizures Febrile Seizures Status Epilepticus Headache

4. Seizures

5. 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

6. 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

7. 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

8. 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

9. 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

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

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

12. 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

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

14. How to differentiate “Staring Spells” 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

15. 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)

16. Epilepsy Syndromes West Syndrome Infantile Spasms Onset in 1 st 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

17. Epilepsy Syndromes 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

18. 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

19. 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

20. 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

21. 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

22. 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

23. Febrile Seizures

24. 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

25. 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.

26. 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

27. 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

28. 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%

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

30. Typical vs Atypical Febrile Seizures 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

31. 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

32. 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

33. 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

34. 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

35. 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.

36. 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

37. 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

38. 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%

39. Status Epilepticus

40. 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

41. 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

42. 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

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

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

45. 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)

46. 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

47. 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

48. 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

49. 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

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

51. Headache

52. Question 1 A 7 year old male presents with headache. Which of the following would NOT be a “red flag” on history? A. Early morning vomiting B. Headache worse after certain foods C. Vomiting without nausea D. Focal neurologic symptoms

53. Question 2 Which is the following is FALSE regarding migraine in children A. The headache can last as little as 1 hour in children B. Children do not need to have nausea AND vomiting to be diagnosed with migraine C. There is often a family history of migraine D. MRI is often needed to rule ot other serious causes of headache.

54. Question 3 Which of the following medications has the best evidence for aborting migraine in children? A. Acetaminophen B. Demerol C. Sumatripan D. Ibuprofen

55. Question 4 Which of the following is NOT a migraine variant in childhood? A. Alice in Wonderland syndrome B. Paroxysmal Torticollis C. Cyclic Vomiting Syndrome D. Benign Paroxysmal Vertigo E. All of the above are migraine variants in childhood

56. Key Questions to ask on H/A Hx Duration Constant or Intermittent Quality of Pain (ie throbbing, pressure) Scale 1-10 Location of pain +/- radiation Nausea or vomitting Photo or Phonophobia Aggravating and Alleviating factors

57. Key Questions to ask on H/A Hx Early am waking Weight loss, fever etc Aura / Visual changes Focal neuro symptoms Change with position / Valsalva Family Hx of H/A

58. Key items on Physical Temperature Blood pressure and CVS exam Cranial Bruits Scalp tenderness Fundi Focal neurological signs

59. H/A in increased ICP Nocturnal or early morning H/A in 15% Nx and Vx in 50% May be precipitated by change in position / Valsalva

60. Other features of Brain Tumours/ H/A in increased ICP Personality change, memory problems, poor concentration Seizures in 1/3 Vomiting NOT preceded by nausea Focal neuro findings Papilledema – formally seen in 60-70% –Now seen in ~ 10-20% –Likely due to better neuroimaging techniques

61. Migraine Epidemiology – 75% of H/A’s referred for pediatric neurologic consultation –prevalence 1.2 – 11% depending on age +ve family hx in 70 – 90%

62. Key Features May have previous history of motion sickness Headache is dull then becomes pulsating/throbbing (NOT maximal at onset) Unilateral (2/3) or bilateral (1/3) Can be associated with cutaneous allodynia

63. Key Features Ask re: nausea, vomiting, anorexia, relief with sleep, “Do they look sick”? Triggers: exercise, anxiety, fatigue, head trauma, menses, foods (chocolate, nitrites, MSG) Auras: visual changes, dysesthesias of limbs and perioral region –For auras, ask re: sudden onset vs gradual onset

64. Diagnostic Criteria A. At least 5 attacks B. Headache lasting 30 min to 48 hrs C. Headache has at least 2 of the following 1.Bilateral (fronto-temporal) or unilateral location 2.Pulsating quality 3.Moderate to severe intensity 4.Aggravation by routine physical activity D. During headache, at least 1 of: –1. Nausea or vomiting –2. Photophobia or phonophobia

65. Migraine Treatment Abortive Reference: Neurology, 2004 Best Evidence (Level A) –Ibuprofen (10mg/kg) Level B –Acetaminophen (15 mg/kg) (Often need to tell parents correct dose) Intranasal Sumatriptan effective in adolescents –(5-20 mg at onset of H/A, can repeat X 1) Insufficient evidence for oral triptans

66. Migraine Variants: With Headache Hemiplegic Migraine Confusional Migraine Basilar Migraine Ophthalmoplegic Migraine

67. Migraine Variants: No Headache Alice in wonderland syndrome Benign Paroxysmal Vertigo Paroxysmal Torticollis Cyclic Vomitting

68. Question 1 A 7 year old male presents with headache. Which of the following would NOT be a “red flag” on history? A. Early morning vomiting B. Headache worse after certain foods C. Vomiting without nausea D. Focal neurologic symptoms

69. Question 2 Which is the following is FALSE regarding migraine in children A. The headache can last as little as 1 hour in children B. Children do not need to have nausea AND vomiting to be diagnosed with migraine C. There is often a family history of migraine D. MRI is often needed to rule ot other serious causes of headache.

70. Question 3 Which of the following medications has the best evidence for aborting migraine in children? A. Acetaminophen B. Demerol C. Sumatripan D. Ibuprofen

71. Question 4 Which of the following is NOT a migraine variant in childhood? A. Alice in Wonderland syndrome B. Paroxysmal Torticollis C. Cyclic Vomiting Syndrome D. Benign Paroxysmal Vertigo E. All of the above are migraine variants in childhood

72. Questions?