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