NEUROLOGY FOR NEUROLOGY FOR NURSESNURSES

ANDREA VAN LIEROP RN BSNArkansas Children’s Hospital

Children with EpilepsyChildren with EpilepsySchool IssuesSchool 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.

DEFINITIONDEFINITION

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.

EpilepsyEpilepsy

Recurrent seizures produced by abnormal repetitive neuronal firing in the brain

Occurs in 1-2% of the population

Seizure ClassificationSeizure Classification

Partial (Focal) Primary Generalized

Simple Complex Absence Myoclonic Atonic Convulsive

Tonic

Clonic

Tonic-clonic

Tonic-clonic-tonic

May secondarily generalize

Evaluation of EpilepsyEvaluation 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 EpilepsyEvaluation 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

General Guidelines for General Guidelines for Therapy: Therapy:

Correct classification of seizures leads to correct AED selection

Treat when the benefit of therapy outweighs the risk

Avoid polypharmacyMonotherapy usually results in better

seizure control and less side effects

General Guidelines for General Guidelines for Therapy:Therapy:

Maximize one medication before changing to the second

Treat the patient, not the EEG or the AED level

Use rational polypharmacy when indicatedIF IT’S NOT BROKEN, DON’T TRY TO

FIX IT!!!!!!!!!!

““Women” of Child Women” of Child Bearing Age on AED’s: 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’sDevelopment of AED’s

1993– Phenobarbital– Other barbiturates

Primidone

– Phenytoin (Dilantin)

– Ethosuxmide (Zarontin)

– Carbamazepine (Tegretol)

– Valproate (Depakote, Depakene)

1993 – Felbamate (Felbatol)

– Gabapentin (Neurontin)

– Lamotrigine (Lamictal)

– Tiagabine (Gabatril)

– Topiramate (Topamax)

– Oxcarbazepine (Trileptal)

– Levetiracetam (Keppra)

– Zonisamide (Zonegran)

NatalieNatalie

3-week-old infant with no apparent perinatal complications

HypotoniaSlow feeder, poor suckSeveral spells per day of staring with slight

jerking movements

Neonatal SeizureNeonatal Seizure

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

SamuelSamuelSamuelSamuel

Infantile SpasmsInfantile Spasms

Infantile SpasmsInfantile 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

HypsarrhythmiaHypsarrhythmiaFp1-F3

F3-C3

C3-P3

P3-O1

Fp2-F4

F4-C4

C4-P4

P4-O2

Fp1-F7

F7-T3

T3-T5

T5-O1

Fp2-F8

F8-T4

T4-T6

T6-O2

1 sec 50 µV

Electrodecremental Seizure

9-year-old boy with long-standing history of intractable epilepsy with tonic-clonic, tonic, myoclonic, atypical absence and atonic seizures

Moderate mental retardationFrequent injuries from falls

CharlieCharlieCharlieCharlie

Head DropsHead Drops

Lennox-Gastaut SyndromeLennox-Gastaut Syndrome

Onset in early childhoodMultiple seizure types including

– Atypical absence, generalized convulsive, atonic, myoclonic, partial

Negative neurodevelopmental impact– Mental retardation

Slow spike and wave (2 hertz)

AtonicAtonic

No warning; abrupt onsetLoss of muscular tone results in sudden fallBrief durationInjuries commonVery difficult to treat

AtonicAtonic

TerryTerry

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 consciousnessLMD felt these could not be seizures

Simple Partial SeizureSimple Partial Seizure

GabrielleGabrielle

12 y/o hispanic girl with 1-year history of frequent spells with several daily

Diagnosed with “pseudoseizures” Maintenance of consciousnessEvents 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 SeizureFrontal Lobe Seizure

Simple PartialSimple Partial

Focal onsetAura is commonNo alteration of consciousnessMay secondarily generalize

WilliamWilliam

12-year-old boy with intractable seizures with episodes of confusion and disorientation

Ash leaf spots noted on Wood’s lamp examIntractable seizures despite numerous

AED’s

Complex Partial SeizureComplex Partial Seizure

Complex PartialComplex Partial

Focal onsetAura is commonAlteration of consciousnessAutomatismsPostictal confusionMay secondarily generalize

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

ChelseaChelseaChelseaChelsea

Bilateral Centrotemporal SpikesBilateral Centrotemporal Spikes

Fp1-F3

F3-C3

C3-P3

P3-O1

fp2=f4

F4-C4

C4-P4

P4-o21 sec

50 µV

Benign Focal Epilepsy of Benign Focal Epilepsy of ChildhoodChildhood

Rolandic (centrotemporal) or occipital spikes

Nocturnal seizures are commonSeizures are usually infrequentRemits by 15-16 years of age or earlier

Benign Focal Epilepsy of Benign Focal Epilepsy of Childhood: TreatmentChildhood: Treatment

*Treatment may not be warranted for uncomplicated, infrequent seizures. If seizures are frequent or tend to secondarily generalize, treatment should be considered.

13 y/o girl with frequent staring episodes described as a blank stare with unresponsiveness, noted daily at school

Decreasing school performanceKey question: Is there an acute arrest of

activity?

TinaTinaTinaTina

AbsenceAbsence

AbsenceAbsence

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: TreatmentAbsence: Treatment

1st Choice AED’s– Ethosuximide (Zarontin)

– Valproate (Depakote,

Depakene)

Phenytoin, Phenobarbital, Carbamazepine, Gabapentin, TopiramateMay even exacerbate seizures

Absence Followed by Absence Followed by Clonic-Tonic-ClonicClonic-Tonic-Clonic

Primary Generalized Primary Generalized ConvulsiveConvulsive

No warning; abrupt onsetTonic, clonic, tonic-clonic or clonic-tonic-

clonic activityBowel and bladder incontinence commonPostictal unresponsiveness or confusionGeneralized spike and wave

Primary Generalized Primary Generalized Convulsive: TreatmentConvulsive: Treatment

1st Choice AED’s– Valproate (Depakote,

Depakene)

Primary Generalized Primary Generalized Convulsive & Absence: Convulsive & Absence: TreatmentTreatment 1st Choice AED’s

– Valproate (Depakote,

Depakene) – Lamotrigine (Lamictal)

2nd Choice AED’s– Topiramate (Topamax)

– Ethosuximide + Valproate

– Zonisamide– Levetiracetam (Keppra)

– Felbamate (Felbatol)

13-year-old boy who had a single generalized convulsion 3 weeks ago

No previous history of seizuresKey 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”

BubbaBubbaBubbaBubba

Myoclonic SeizureMyoclonic Seizure

MyoclonicMyoclonic

Sudden single jerks of the arms and headMay occur in clustersNo alteration in consciousnessMay progress to a clonic-tonic-clonic

seizureGeneralized multispike waveValproate, Clonazepam, Zonisamide

Juvenile Myoclonic Epilepsy: Juvenile Myoclonic Epilepsy: TreatmentTreatment

1st Choice AED’s

– Valproate (Depakote,

Depakene)

Juvenile Myoclonic EpilepsyJuvenile 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 DrugThe 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

MADMAD

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 911WHEN 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 Alternative Therapy for EpilepsyEpilepsy

Ketogenic diet

Vagus nerve stimulator

Epilepsy surgery

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 DietKetogenic Diet

Ketogenic Diet: EfficacyKetogenic Diet: Efficacy

150 children prospectively evaluatedAge range: 4 months - 16 yearsAverage of 410 seizures per monthResults 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:Vagus Nerve Stimulator:Rule of ThirdsRule 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:Epilepsy Surgery:Criteria for ConsiderationCriteria for Consideration

Seizures must be medically intractable

Seizures must be debilitating

There should be no chance for spontaneous resolution

Epilepsy Epilepsy SurgerySurgery Temporal lobectomy

– 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

14 year old girl with 1 week history of new onset convulsive seizures

Hospitalized and loaded with phenytoin with worsening seizures

Normal EEG

MonicaMonicaMonicaMonica

Is it Real or is it Memorex?Is it Real or is it Memorex?

Pseudoseizure

#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

NES in Children:NES in Children:Psychosocial StressorsPsychosocial Stressors

CASE HISTORYCASE 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 2Case 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 3Case 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?ANY QUESTIONS?