1. LMCC Review: Pediatric Neurology
Asif Doja, MD, FRCP(C)
April 3rd, 2008

2. Outline Seizures Febrile Seizures Status Epilepticus
Hypotonia in the Newborn and Cerebral Palsy

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

13. 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

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. Epilepsy Syndromes 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

16. 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

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 %
D. 33%

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

29. Typical vs Atypical Febrile Seizures
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

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 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 %
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. Hypotonia and Cerebral Palsy

51. Question 1

53. The child in the preceding picture is alert, has little spontaneous movement and no reflexes. He most likely has:
A. Central Hypotonia
B. Peripheral Hypotonia
C. Mixed Central and Peripheral Hypotonia
D. None of the above

54. Question 2
Which of the following on obstetrical history is NOT usually associated with hypotonia in the newborn?
A. Decreased fetal movement
B. Breech presentation
C. Jitteriness immediately after birth
D. Polyhydramnios

55. Question 3
The following would all be considered causes of peripheral hypotonia EXCEPT:
A. Spinal Muscular Atrophy
B. Neonatal Myasthenia Gravis
C. Myotonic Dystrophy
D. Trisomy 21

56. Question 4
With respect to Cerebral Palsy, which of the following statements is NOT correct?
A. 66% of cases are due to intrapartum asphyxia
B. Prematurity is a leading cause of the spastic diplegic form
C. in 1/3 of cases there is no etiology
D. A majority of patients have spasticity

57. Question 5
Which of the following is the most common form of cerebral palsy?
A. Spastic
B. Athetoid/Dystonic
C. Ataxic
D. Mixed

58. Hypotonia Decreased resistance to movement “floppy baby”
Obstetrical/Perinatal History
Fetal movement
Breech presentation
Polyhydramnios
History of miscarriage
Resuscitation at birth

59. Hypotonia
Check for resting posture “frog legged position” (indicates peripheral)
Check infant’s postural tone
Traction response – pull to sit
Vertical (axillary) suspension
Horizontal (ventral) suspension

60. Hypotonia Central “Floppy and Strong” preserved reflexes
May not be alert
Peripheral
“Floppy and Weak”
absent reflexes
alert

61. Hypotonia - Central Genetic Perinatal Problems Infections
Trisomy 21, Prader-Willi
Perinatal Problems
Perinatal asphyxia
Infections
TORCH

62. Hypotonia - Peripheral
Motor Neuron
Spinal Muscular Atrophy (SMA)
Peripheral Nerve
Neuromuscular junction
Neonatal mysathenia gravis
Muscle
Congenital muscular dystrophy, myotonic dystrophy

63. Cerebral Palsy
Nonprogressive impairment of central motor function, caused by insult or anomaly of the immature CNS
Only 10% due to intrapartum asphyxia
No etiology in 1/3 of cases
Can present with low tone initially then with spastic tone
Velocity dependent increase in resistance to movement

64. Cerebral Palsy- Types Spastic 70-80%
Can be spastic diplegia in prems (from periventricular leukomalacia)
Quadriplegia with hypoxic-ischemic encephalopathy (asphyxia)

65. Cerebral Palsy- Types Athetoid/Dystonic Ataxic Mixed 10-15%
Due to damage to basal ganglia
Ataxic
<5%
Damage to cerebellum or thalamus
Mixed

66. Question 1

68. The child in the preceding picture is alert, has little spontaneous movement and no reflexes. He most likely has:
A. Central Hypotonia
B. Peripheral Htypotonia
C. Mixed Central and Peripheral Hypotonia
D. None of the above

69. Question 2
Which of the following on obstetrical history is NOT usually associated with hypotonia in the newborn?
A. Decreased fetal movement
B. Breech presentation
C. Jitteriness immediately after birth
D. Polyhydramnios

70. Question 3
The following would all be considered causes of peripheral hypotonia EXCEPT:
A. Spinal Muscular Atrophy
B. Neonatal Myasthenia Gravis
C. Myotonic Dystrophy
D. Trisomy 21

71. Question 4
With respect to Cerebral Palsy, which of the following statements is NOT correct?
A. 66% of cases are due to intrapartum asphyxia
B. Prematurity is a leading cause of the spastic diplegic form
C. in 1/3 of cases there is no etiology
D. A majority of patients have spasticity

72. Question 5
Which of the following is the most common form of cerebral palsy?
A. Spastic
B. Athetoid/Dystonic
C. Ataxic
D. Mixed

73. Questions?