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 Occurs in 1-2% of the population

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

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 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 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 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)

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 80% develop mental retardation 60-70% have lifelong epilepsy Hypsarrhythmia pattern on EEG

Hypsarrhythmia Electrodecremental Seizure

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

Head Drops

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)

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 Focal onset Aura is common No alteration of consciousness May secondarily generalize

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

Complex Partial Seizure

Complex Partial 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

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 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-tonic-clonic 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 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 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 Seizures must be debilitating There should be no chance for spontaneous resolution

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

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 #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?