1. NEUROLOGY FOR NURSES ANDREA VAN LIEROP RN BSN
Arkansas Children’s Hospital

2. Children with Epilepsy School Issues
Children with poor seizure control are more likely to have trouble making friends.
Taking medication at school may be associated with a significant decrease in social and peer relationships. Even in children with self reported good seizure control.

3. DEFINITION
A seizure is a sudden and stereotyped alteration in motor activity, sensation, behavior or consciousness due to an abnormal electrical discharge of neurons
Epilepsy is a chronic neurological condition characterized by recurrent, unprovoked seizures.

4. Epilepsy
Recurrent seizures produced by abnormal repetitive neuronal firing in the brain
Occurs in 1-2% of the population

5. Seizure Classification
Partial (Focal) Primary Generalized
Simple Complex Absence Myoclonic Atonic Convulsive
Tonic
Clonic
Tonic-clonic
Tonic-clonic-tonic
May secondarily generalize

6. Evaluation of Epilepsy
EEG & Epilepsy
Awake only ( 30-40% abnormal)
Awake and asleep ( 60-70% abnormal)
Photic stimulation
may induce generalized spike and wave or occipital spikes
Hyperventilation
may induce 3/sec. spike and wave (absence)

7. Evaluation of Epilepsy
Neuroimaging
Indicated with:
Abnormal neurological exam
Focal onset seizures
Uncertain if focal or primary generalized onset
Onset of seizures after adolescence
MRI is the gold standard (not CT)
CT is helpful in the acute setting
i.e. persistent alteration of consciousness or abnormal neurologic exam

8. General Guidelines for Therapy:
Correct classification of seizures leads to correct AED selection
Treat when the benefit of therapy outweighs the risk
Avoid polypharmacy
Monotherapy usually results in better seizure control and less side effects

9. General Guidelines for Therapy:
Maximize one medication before changing to the second
Treat the patient, not the EEG or the AED level
Use rational polypharmacy when indicated
IF IT’S NOT BROKEN, DON’T TRY TO FIX IT!!!!!!!!!!

10. “Women” of Child Bearing Age on AED’s:
All should receive folic acid supplementation with a minimum of 1mg/day, or 4mg/day if they are pregnant or actively trying to become pregnant

11. Development of AED’s 1993 1993 Felbamate (Felbatol) Phenobarbital
Gabapentin (Neurontin)
Lamotrigine (Lamictal)
Tiagabine (Gabatril)
Topiramate (Topamax)
Oxcarbazepine (Trileptal)
Levetiracetam (Keppra)
Zonisamide (Zonegran)
1993
Phenobarbital
Other barbiturates
Primidone
Phenytoin (Dilantin)
Ethosuxmide (Zarontin)
Carbamazepine (Tegretol)
Valproate (Depakote, Depakene)

12. Natalie 3-week-old infant with no apparent perinatal complications
Hypotonia
Slow feeder, poor suck
Several spells per day of staring with slight jerking movements

13. Neonatal Seizure

14. Samuel
7-month-old infant with cerebral injury from “shaken baby” syndrome
Now with episodes described as “startle reflexes” commonly occurring in clusters
Not responsive to maintenance phenobarbital

15. Infantile Spasms

16. Infantile Spasms Flexion or extension spasms Tend to occur in clusters
Cryptogenic vs. symptomatic
Many potential causes
Onset: Birth to 2 years
Peak onset: 5-6 months
80% develop mental retardation
60-70% have lifelong epilepsy
Hypsarrhythmia pattern on EEG

17. Hypsarrhythmia
Electrodecremental
Seizure

18. Charlie
9-year-old boy with long-standing history of intractable epilepsy with tonic-clonic, tonic, myoclonic, atypical absence and atonic seizures
Moderate mental retardation
Frequent injuries from falls

19. Head Drops

20. Lennox-Gastaut Syndrome
Onset in early childhood
Multiple seizure types including
Atypical absence, generalized convulsive, atonic, myoclonic, partial
Negative neurodevelopmental impact
Mental retardation
Slow spike and wave (2 hertz)

21. Atonic No warning; abrupt onset
Loss of muscular tone results in sudden fall
Brief duration
Injuries common
Very difficult to treat

22. Atonic

23. Terry
2-year-old boy with 3-week history of recurrent episodes of sudden fear or panic
Initial frequency of 2-3 per day, now increased to 1 per hour or more
Maintenance of consciousness
LMD felt these could not be seizures

24. Simple Partial Seizure

25. Gabrielle
12 y/o hispanic girl with 1-year history of frequent spells with several daily
Diagnosed with “pseudoseizures”
Maintenance of consciousness
Events are stereotypical with extension of the right arm and turning to the left, followed by agitated movements and vocalization with an abrupt recovery

26. Frontal Lobe Seizure

27. Simple Partial Focal onset Aura is common
No alteration of consciousness
May secondarily generalize

28. William
12-year-old boy with intractable seizures with episodes of confusion and disorientation
Ash leaf spots noted on Wood’s lamp exam
Intractable seizures despite numerous AED’s

29. Complex Partial Seizure

30. Complex Partial Focal onset Aura is common Alteration of consciousness
Automatisms
Postictal confusion
May secondarily generalize

31. Chelsea 9-year-old girl with 2 seizures during the last 4 months
First episode occurred during sleep consisting of a brief generalized convulsion
Second seizure occurred during wakefulness and involved the right face and arm with subsequent secondary generalization

32. Bilateral Centrotemporal Spikes

33. Benign Focal Epilepsy of Childhood
Rolandic (centrotemporal) or occipital spikes
Nocturnal seizures are common
Seizures are usually infrequent
Remits by years of age or earlier

34. Benign Focal Epilepsy of Childhood: Treatment
*Treatment may not be warranted for uncomplicated, infrequent seizures.
If seizures are frequent or tend to secondarily generalize, treatment should be considered.

35. Tina
13 y/o girl with frequent staring episodes described as a blank stare with unresponsiveness, noted daily at school
Decreasing school performance
Key question: Is there an acute arrest of activity?

36. Absence

37. Absence Brief staring episodes with unresponsiveness
Sudden onset with an arrest of activity
No aura or postictal confusion
May have associated eye flutter or simple automatisms
Generalized 3 per second spike and wave
80% will have resolution with age
20% also have convulsive seizures.

38. Absence: Treatment 1st Choice AED’s Ethosuximide (Zarontin)
Valproate (Depakote, Depakene)
Phenytoin, Phenobarbital, Carbamazepine, Gabapentin, Topiramate
May even exacerbate seizures

39. Absence Followed by Clonic-Tonic-Clonic

40. Primary Generalized Convulsive
No warning; abrupt onset
Tonic, clonic, tonic-clonic or clonic-tonic-clonic activity
Bowel and bladder incontinence common
Postictal unresponsiveness or confusion
Generalized spike and wave

41. Primary Generalized Convulsive: Treatment
1st Choice AED’s
Valproate (Depakote, Depakene)

42. Primary Generalized Convulsive & Absence: Treatment
1st Choice AED’s
Valproate (Depakote, Depakene)
Lamotrigine (Lamictal)
2nd Choice AED’s
Topiramate (Topamax)
Ethosuximide + Valproate
Zonisamide
Levetiracetam (Keppra)
Felbamate (Felbatol)

43. Bubba
13-year-old boy who had a single generalized convulsion 3 weeks ago
No previous history of seizures
Key question: Do you ever have small jerks of your arms, especially early in the morning?
Answer: “Oh yeah, I’ve been doing that for a couple of years”

44. Myoclonic Seizure

45. Myoclonic Sudden single jerks of the arms and head
May occur in clusters
No alteration in consciousness
May progress to a clonic-tonic-clonic seizure
Generalized multispike wave
Valproate, Clonazepam, Zonisamide

46. Juvenile Myoclonic Epilepsy: Treatment
1st Choice AED’s
Valproate (Depakote, Depakene)

47. Juvenile Myoclonic Epilepsy
Autosomal dominant inheritance
Chromosome 6
Myoclonic seizures with onset in late childhood or adolescence
May develop generalized convulsive or absence seizures

48. The Rescue Drug
Diastat Acudial is a diazapam rectal gel intended for the management of seizure patients who require intermittent use of Diazepam for seizure activity greater than 5 min.
10mg diastat Acudial can be dialed to 5mg 7.5mg or 10mg
20mg Diastat Acudial can be dialed to 12.5mg 15mg 17.5mg and 20mg
2.5mg diastat is still available

49. MAD Nasal Drug Delivery System Fast and Effective Controlled Delivery
No Needles
Midazolam/ Versed
Dosage .2mg/Kg up to max of 10mg
50kg = 10mg/2ml
Deliver slowly in one side of nostril,
hold other side closed

50. WHEN DO YOU CALL 911
If a patient has been given Diastat or versed and is not recovering after 3 min.
If the patient has turned cyanotic and is not breathing
If the patient is having cycles of seizures even after receiving rescue medication.

51. Alternative Therapy for Epilepsy
Ketogenic diet
Vagus nerve stimulator
Epilepsy surgery

52. Ketogenic Diet First described by Wilder, Mayo Clinic Bulletin, 1921
Mark 9:29 “This kind can come forth by nothing, but by prayer and fasting.”
Typically reserved for children with severe, debilitating and intractable seizures
4:1 (Fat: Protein + Carbohydrates)
75-90% of caloric intake as fat
Urinary ketosis millimolar

53. Ketogenic Diet: Efficacy
150 children prospectively evaluated
Age range: 4 months - 16 years
Average of 410 seizures per month
Results after 1 year:
55% remained on the diet
1/2 had > 50% reduction in seizures
1/4 had > 90% decrease in seizures
Freeman, Vining, et.al. Pediatrics, December, 1998

54. Vagus Nerve Stimulator: Rule of Thirds
1/3 - marked improvement
1/3 - some improvement
1/3 – little/no improvement
Potential benefits
fewer seizures, less severe seizures, shorter recovery period, decreased meds and side effects, less fear and anxiety, more control

55. Epilepsy Surgery: Criteria for Consideration
Seizures must be medically intractable
Seizures must be debilitating
There should be no chance for spontaneous resolution

56. Epilepsy Surgery Temporal lobectomy Extratemporal lesional resection
75-90% seizure free
Extratemporal lesional resection
50-75% seizure free
Extratemporal non-lesional resection
< 50% seizure free
Functional hemispherectomy
Corpus callosotomy
Especially for atonic and brief tonic seizures

57. Monica
14 year old girl with 1 week history of new onset convulsive seizures
Hospitalized and loaded with phenytoin with worsening seizures
Normal EEG

58. Is it Real or is it Memorex?
Pseudoseizure

59. NES in Children: Psychosocial Stressors
#1 Sexual or physical abuse
Others:
Death of a family member
Forced separation from family members
Physical disability or illness of a parent
Significant family conflict
Major illness
Financial stressors
Moving
Relational difficulties

60. CASE HISTORY
An 8 year old girl in 3rd grade has recently been noted to have staring episodes at school. She also has a recent decline in grades.

61. Case history 2
A 9th grader has a generalized convulsion in the school lunch room. He had been very stressed with exams and was extremely tired. Upon questioning you learn that he also has jerks of his arms, especially in the morning.

62. Case history 3 A 6 year old has experienced 2 seizures.
The first occurred 3 months ago during sleep. His parents took him to the ER. His CT and labs were normal. He was scheduled for an outpatient EEG but did not keep apt. His second seizure happened during nap time, his kindergarten teacher noticed he had initial twitching of the right face and arm followed by a brief generalized convulsion.

63. ANY QUESTIONS?