Transcript Epilepsy in Children

NEUROLOGY FOR
NURSES
ANDREA VAN LIEROP RN BSN
Arkansas Children’s Hospital
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.
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.
Epilepsy

Recurrent seizures produced by abnormal
repetitive neuronal firing in the brain
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Occurs in 1-2% of the population
Seizure Classification
Partial (Focal)
Simple
Complex
Primary Generalized
Absence Myoclonic Atonic Convulsive
Tonic
May secondarily generalize
Clonic
Tonic-clonic
Tonic-clonictonic
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)
Evaluation of Epilepsy
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Neuroimaging
– Indicated with:
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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
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
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!!!!!!!!!!
“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
Development of AED’s

1993

1993
– Phenobarbital
– Felbamate (Felbatol)
– Other barbiturates
– Gabapentin (Neurontin)
Primidone
– Lamotrigine (Lamictal)

– Phenytoin (Dilantin)
– Tiagabine (Gabatril)
– Ethosuxmide (Zarontin)
– Topiramate (Topamax)
– Carbamazepine (Tegretol)
– Oxcarbazepine (Trileptal)
– Valproate (Depakote,
– Levetiracetam (Keppra)
Depakene)
– Zonisamide (Zonegran)
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
Neonatal Seizure
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
Infantile Spasms
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
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80% develop mental retardation
 60-70% have lifelong epilepsy
 Hypsarrhythmia pattern on EEG
Hypsarrhythmia
Electrodecremental
Seizure
Fp1-F3
F3-C 3
C 3-P3
P3-O1
Fp2-F4
F4-C 4
C 4-P4
P4-O2
Fp1-F7
F7-T3
T3-T5
T5-O1
Fp2-F8
F8-T4
T4-T6
T6-O2
1 s ec
50 µV
Charlie
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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
Head Drops
Lennox-Gastaut Syndrome
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Onset in early childhood
 Multiple seizure types including
– Atypical absence, generalized convulsive,
atonic, myoclonic, partial
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Negative neurodevelopmental impact
– Mental retardation

Slow spike and wave (2 hertz)
Atonic

No warning; abrupt onset
 Loss of muscular tone results in sudden fall
 Brief duration
 Injuries common
 Very difficult to treat
Atonic
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
Simple Partial Seizure
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
Frontal Lobe Seizure
Simple Partial
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Focal onset
 Aura is common
 No alteration of consciousness
 May secondarily generalize
William
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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
Complex Partial Seizure
Complex Partial
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Focal onset
 Aura is common
 Alteration of consciousness
 Automatisms
 Postictal confusion
 May secondarily generalize
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
Bilateral Centrotemporal Spikes
Fp1-F3
F3-C 3
C 3-P3
P3-O1
fp2=f4
F4-C 4
C 4-P4
50 µV
1 s ec
P4-o2
Benign Focal Epilepsy of
Childhood

Rolandic (centrotemporal) or occipital
spikes
 Nocturnal seizures are common
 Seizures are usually infrequent
 Remits by 15-16 years of age or earlier
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.
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?
Absence
Absence
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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.
Absence: Treatment

1st Choice AED’s
– Ethosuximide (Zarontin)
– Valproate (Depakote,
Depakene)
Phenytoin, Phenobarbital, Carbamazepine, Gabapentin, Topiramate
May even exacerbate seizures
Absence Followed by
Clonic-Tonic-Clonic
Primary Generalized
Convulsive

No warning; abrupt onset
 Tonic, clonic, tonic-clonic or clonic-tonicclonic activity
 Bowel and bladder incontinence common
 Postictal unresponsiveness or confusion
 Generalized spike and wave
Primary Generalized
Convulsive: Treatment

1st Choice AED’s
– Valproate (Depakote,
Depakene)
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)
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”
Myoclonic Seizure
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
Juvenile Myoclonic Epilepsy:
Treatment

1st Choice AED’s
– Valproate (Depakote,
Depakene)
Juvenile Myoclonic Epilepsy

Autosomal dominant inheritance
– Chromosome 6

Myoclonic seizures with onset in late
childhood or adolescence
 May develop generalized convulsive or
absence seizures
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
MAD
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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
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.
Alternative Therapy for
Epilepsy

Ketogenic diet

Vagus nerve stimulator
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Epilepsy surgery
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 80- 160 millimolar
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
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
Epilepsy Surgery:
Criteria for Consideration

Seizures must be medically intractable
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Seizures must be debilitating
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There should be no chance for spontaneous
resolution
Epilepsy
Surgery

Temporal lobectomy
– 75-90% seizure free
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Extratemporal lesional
resection
– 50-75% seizure free
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Extratemporal nonlesional resection
– < 50% seizure free


Functional
hemispherectomy
Corpus callosotomy
– Especially for atonic and
brief tonic seizures
Monica

14 year old girl with 1 week history of new
onset convulsive seizures
 Hospitalized and loaded with phenytoin
with worsening seizures
 Normal EEG
Is it Real or is it Memorex?
Pseudoseizure
NES in Children:
Psychosocial Stressors

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#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
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.
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.
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.
ANY QUESTIONS?