NEUROLOGY FOR NURSES

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NEUROLOGY FOR NURSES
ANDREA VAN LIEROP RN BSN Arkansas Childrens
Hospital
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Children with EpilepsySchool 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.

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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.

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Epilepsy

• Recurrent seizures produced by abnormal
  repetitive neuronal firing in the brain
• Occurs in 1-2 of the population

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

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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

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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

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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 ITS NOT BROKEN, DONT TRY TO FIX IT!!!!!!!!!!

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Women of Child Bearing Age on AEDs

• 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

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Development of AEDs

• 1993
• Felbamate (Felbatol)
• 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)

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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

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Neonatal Seizure
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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

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Infantile Spasms
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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

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Hypsarrhythmia
Electrodecremental Seizure
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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

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Head Drops
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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)

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Atonic

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

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Atonic
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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

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Simple Partial Seizure
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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

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Frontal Lobe Seizure
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Simple Partial

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

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William

• 12-year-old boy with intractable seizures with
  episodes of confusion and disorientation
• Ash leaf spots noted on Woods lamp exam
• Intractable seizures despite numerous AEDs

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Complex Partial Seizure
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Complex Partial

• Focal onset
• Aura is common
• Alteration of consciousness
• Automatisms
• Postictal confusion
• May secondarily generalize

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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

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Bilateral Centrotemporal Spikes
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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

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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.
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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?

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Absence
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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.

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Absence Treatment

• 1st Choice AEDs
• Ethosuximide (Zarontin)
• Valproate (Depakote, Depakene)

Phenytoin, Phenobarbital, Carbamazepine,
Gabapentin, Topiramate May even exacerbate
seizures
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Absence Followed by Clonic-Tonic-Clonic
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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

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Primary Generalized Convulsive Treatment

• 1st Choice AEDs
• Valproate (Depakote, Depakene)

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Primary Generalized Convulsive Absence
Treatment

• 1st Choice AEDs
• Valproate (Depakote, Depakene)
• Lamotrigine (Lamictal)

• 2nd Choice AEDs
• Topiramate (Topamax)
• Ethosuximide Valproate
• Zonisamide
• Levetiracetam (Keppra)
• Felbamate (Felbatol)

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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, Ive been doing that for a
  couple of years

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Myoclonic Seizure
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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

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Juvenile Myoclonic Epilepsy Treatment

• 1st Choice AEDs
• Valproate (Depakote, Depakene)

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Juvenile Myoclonic Epilepsy

• Autosomal dominant inheritance
• Chromosome 6
• Myoclonic seizures with onset in late childhood
  or adolescence
• May develop generalized convulsive or absence
  seizures

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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

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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

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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.

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Alternative Therapy for Epilepsy

• Ketogenic diet
• Vagus nerve stimulator
• Epilepsy surgery

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Ketogenic Diet

• First described by Wilder, Mayo Clinic Bulletin,
  1921
• Mark 929 This kind can come forth by nothing,
  but by prayer and fasting.
• Typically reserved for children with severe,
  debilitating and intractable seizures
• 41 (Fat Protein Carbohydrates)
• 75-90 of caloric intake as fat
• Urinary ketosis 80- 160 millimolar

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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 gt 50 reduction in seizures
• 1/4 had gt 90 decrease in seizures

Freeman, Vining, et.al. Pediatrics, December, 1998
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Vagus Nerve StimulatorRule 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

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Epilepsy SurgeryCriteria for Consideration

• Seizures must be medically intractable
• Seizures must be debilitating
• There should be no chance for spontaneous
  resolution

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Epilepsy Surgery

• Temporal lobectomy
• 75-90 seizure free
• Extratemporal lesional resection
• 50-75 seizure free
• Extratemporal non-lesional resection
• lt 50 seizure free
• Functional hemispherectomy
• Corpus callosotomy
• Especially for atonic and brief tonic seizures

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Monica

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

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Is it Real or is it Memorex?
Pseudoseizure
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NES in ChildrenPsychosocial 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

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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.

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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.

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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.

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ANY QUESTIONS?