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The Whole Child: Conditions Associated with Pediatric Epilepsy. Beverly Wical, MD Gillette Children’s Specialty Healthcare November, 2010. Things we think about at the beginning:. At the beginning…. Identifying cause Control of seizures. As time goes on:. Seizure control

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the whole child conditions associated with pediatric epilepsy

The Whole Child: Conditions Associated with Pediatric Epilepsy

Beverly Wical, MD

Gillette Children’s Specialty Healthcare

November, 2010

at the beginning
At the beginning…
  • Identifying cause
  • Control of seizures
as time goes on
As time goes on:
  • Seizure control
  • Adverse effects of therapy
  • Associated conditions
associated conditions are associated with
Associated conditions are associated with….?
  • Underlying neurologic diagnosis: what causes the epilepsy (and the other problems)
  • Epileptic phenomena
    • Seizure effects
    • Epileptic encephalopathy
  • Medication side effects
underlying neurologic diagnosis the cause of the epilepsy
Underlying neurologic diagnosis: the cause of the epilepsy:
  • Strongest predictor of associated conditions
  • Tells us “where to look” for potential problems
  • Gives clues about a child’s future from experiences of children with similar disorders
underlying cause examples
Underlying cause: examples…
  • Brain injury from prematurity
  • Brain malformation
  • Infection
  • Stroke
  • Trauma
  • Metabolic disorder
direct effects
“Direct effects”
  • Seizure effects
    • Injury during a seizure
    • Sleepiness or headache after seizure
  • Epileptic encephalopathy
    • Epileptiform EEG abnormalities contribute to a progressive disturbance in cerebral function (ILAE)…changes in the EEG change the child’s function
medication side effects
Medication side effects:
  • Very common
  • Often missed
  • The one we can most easily alter…and one caused by us.
  • ALL associated condtions:

COULD IT BE A MEDICATION SIDE EFFECT??

how to screen for associated conditions quality of life
How to screen for associated conditions: Quality of Life…
  • What aspects of the child’s life present the greatest barrier to:
    • Best function in his/her family, work (school) and social groups?
    • Greatest comfort? Physically, emotionally, mentally…
cognition thinking and learning
Cognition: thinking and learning
  • Intellectual ability “IQ”
  • Ways of thinking and learning:
    • Nonverbal…right hemisphere
    • Language based; expressive/receptive…left hemisphere
  • Executive functioning…frontal lobes
  • Memory…temporal (and frontal) lobes
  • Visual/spatial/motor (right hemisphere, parietal lobes)
slide15
Mood
  • Anxiety
  • Depression
  • Irritability
  • Mood swings
behavior
Behavior
  • Oppositional
  • Irritable
  • Angry
  • Aggressive
  • Too passive, “not interested”
  • Hyperactive
  • Autism spectrum disorder
sleep
Sleep
  • Excessive daytime sleepiness
  • Insomnia
  • Sleep disordered breathing
  • Delayed sleep phase
  • Behavioral
  • Seizure related sleep disruption
autonomic function
Autonomic function
  • “Automatic” functions
    • Heart rate
    • Breathing rate/control
    • Blood pressure
    • Body temperature (core, extremities)
    • Sweating
    • Pupillary control
    • Gastrointestinal function
    • Bladder function
food stuff
Food stuff…
  • Appetite
  • Weight gain or loss
  • Abdominal discomfort, nausea, vomiting
  • Feeding intolerance, delayed gastric emptying
motor impairments
Motor impairments
  • Developmental coordination disorder
  • Low muscle tone (hypotonia)
  • Balance impairments
  • Cerebral palsy
    • Quadriplegia (all)
    • Hemiplegia (half)
    • Diplegia (both legs)
bone health
Bone health:
  • Assessment for reduced bone density, risk for fractures
  • Related to use of certain AEDs:
    • Phenobarbital
    • Phenytoin
    • Carbamazepine
    • Valproate
    • Ketogenic diet
    • Polypharmacy
  • Much information on children remains unknown
bone health risks may be additive
Bone health: risks may be additive:
  • Ethnicity (white, Asian)
  • Family hx of osteoporosis
  • Inability to bear weight/walk
  • Poor nutrition; vit D or calcium deficiency
  • Hyperthyroidism, hyperparathyroidism
  • Liver disease
  • Medications: steroids
bone health1
Bone health:
  • Enough vitamin D? (600 – 800 IU/d)
  • Enough calcium? (1000 – 1200 mg/d)
  • Increased risk of low bone density
    • Fracture risk?
sally 10 yo girl
Sally: 10 yo girl
  • Focal seizures
  • Gasp, stiffen, turn head to R, jerking right arm
  • Brief episodes (all less than 2 min)
  • All in sleep (night time, occasional in nap)
slide29
Abnormal EEG, left frontal region
  • Normal MRI
  • No other health problems identified
  • Seizure suspected to come from L frontal lobe
sally associated problems to screen for
Cognition

Mood

Behavior

Sleep

Autonomic

Food

Motor

Bone health

Other

Sally: associated problems to screen for…
frontal lobe function
Frontal lobe function:
  • Emotional control center
  • Attention
  • Impulse control
  • Judgment
frontal lobe function1
Frontal lobe function:
  • Problem solving
  • Mental flexibility
    • Can’t switch tasks back and forth
    • Gets “stuck” on one thing
  • Interpreting feedback from situation to guide one’s behavior (making someone mad, backing down verbally/walking away)
  • External cues to guide one’s behavior (when to sit and stand visiting a church/ forks at fancy dinner)
sally cognition learning
Sally: cognition/learning:
  • Getting B’s and C’s in 4th grade
  • Struggles with attention, often cannot “stay on track,” gets frustrated easily
sally mood behavior
Sally: mood, behavior:
  • Irritable, “moody” –worse last few months
  • Very anxious about social situations and “tests” at school
sally assessment of frontal lobe function
Sally: assessment of frontal lobe function
  • Neuropsychology:
    • IQ 97
    • Meets criteria for anxiety disorder
    • Impaired working memory, attention and impulse control.
    • Does not need stimulant medication. Special adaptations in class room suggested.
  • Psychology:
    • strategies to address anxiety; involvement in social skills group
  • Psychiatry…can be very helpful, as brain and mind in same place!
sally mood medication adverse effect
Sally: Mood MEDICATION ADVERSE EFFECT?
  • Started new medication 4 mo ago…irritability worse!
  • Lamotrigine and phenytoin most mood neutral…Most AEDs can do this!!
  • Plan to transition to lamotrigine
sally sleep assessment
Sally: sleep assessment
  • Bed time?
  • Bedtime routine, sleep location?
  • Falls asleep ok? Insomnia?
  • Hours slept/night?
  • Restless sleeper?
  • Seizures disturbing sleep?
signs of sleep disordered breathing
Signs of sleep disordered breathing:
  • Snores? (loud, always, half the time?)
  • Struggles to breathe at night?
  • Ever stop breathing at night?
  • Hard to wake up in morning?
  • Breathe through mouth in day?
  • Headaches upon awaking?
  • Occasionally wet the bed?
  • Is over weight?
  • Have signs/symptoms of ADHD?
slide39
Wake up unrefreshed?
  • Hard to wake up?
  • Too sleepy in the daytime?
sally s sleep
Sally’s sleep:
  • Often anxious at bedtime
  • Trouble falling asleep
  • Positive SDB review:
    • Snores most of the time
    • Breathes heavily in sleep
    • Breathes with mouth open in daytime
    • Often sleepy at school
    • Poor attention, easily distractible
sally s sleep1
Sally’s sleep
  • Referral to pediatric sleep specialist
    • Large tonsils on exam!
  • Polysomnogram (sleep study) performed with simultaneous Video EEG
    • Obstructive sleep apnea
    • Seizure captured in sleep did not cause additional apnea, but did wake her up
  • Referral to ENT: tonsillectomy?
sally s food
Sally’s food:
  • Sally’s appetite is increased significantly in the last 3 months. She has gained 12 pounds. She is “hungry all the time.”
  • New AED started 4 mo ago
sally s food medication side effect gain
Sally’s food: MEDICATION SIDE EFFECT?…gain
  • Oxcarbazepine (carbamazepine too)
  • Pregabalin (also gabapentin)
  • Valproate
  • Others…
  • Medication transition initiated: switch to lamotrigine beginning
sally s food other causes of weight gain
Sally’s food: other causes of weight gain
  • Obstructive sleep apnea
  • Anxiety/stress
  • Life style considerations
    • Family food habits
    • Exercise
sally s motor
Sally’s Motor:
  • Always “a little clumsy”
  • Left handed
  • Slow, poor handwriting
sally s motor1
Sally’s motor:
  • Occupational therapy evaluation:
    • Slowed fine motor skills, R > L
    • Difficulty coordinating bilateral finger movements
    • Would benefit from adaptive writing tools at school; should learn keyboarding as soon as possible
    • Allowing her to “show what she knows” without timed writing exercises would be of benefit
changes in sally s care plan due to associated condition identification and management
Changes in Sally’s care plan due to associated condition identification and management:
  • Cognition/learning
    • Attention and impulse control issues identified; school programming changed to accommodate
  • Mood and behavior
    • Anxiety disorder diagnosed; treatment with therapist and social skills group initiated
    • Irritability at baseline recognized as part of frontal lobe dysfunction. Worsened by current AED therapy, medication switch initiated.
slide48
Sleep
    • Obstructive sleep apnea diagnosed; tonsillectomy resolved problem. Better sleep at night, less daytime sleepiness, less irritable, better attention.
  • Weight gain
    • Due largely to AED; medication switch initiated. Exercise program started, also reduces anxiety.
  • Motor
    • Identification of fine motor problems has led to adaptations in classroom
joey 6 yo boy
Joey: 6 yo boy
  • Tonic seizures:
    • Stiffens all over, L body posture different than R
  • Focal seizures
    • Stop activity, change facial expression, stare, turn head and eyes to L ; ~ 30s
  • Secondarily generalized seizures
    • May or may not start with a recognizable focal seizure; stiffening and shaking of all 4 extremities
slide50
Joey:
  • Born at 25 weeks gestation
  • Brain injury due to prematurity
  • Cerebral palsy; both legs (diplegia) and L arm/hand
slide51
Joey:
  • EEG: multifocal spikes; R temporal and frontal; spikes generalize in sleep to both sides
  • MRI: periventricular leukomalacia; worse in R hemisphere
joey associated conditions to screen
Joey: Associated conditions to screen
  • Cognition
  • Mood
  • Behavior
  • Sleep
  • Autonomic
  • Food
  • Motor
  • Bone health
  • Other
joey cognition and learning
Joey: cognition and learning
  • Has IEP in place
  • In Kindergarten
  • Mother thinks he is “smart”; has never been formally assessed
joey cognition learning
Joey: cognition/learning
  • Neuropsychology evaluation:
  • Speech impairment; normal receptive language, verbal IQ likely >90
  • Non verbal learning disability
    • Performance IQ 65
    • Visual spatial/visual motor impairments
    • Mild attention impairments
joey mood and behavior
Joey: mood and behavior
  • No problems identified
  • “what to watch for”
    • Anxiety
    • Rigidity of thought
    • Attention problems
    • **Social problems related to non verbal learning impairments
joey sleep
Joey: sleep
  • Snores at night
  • Restless sleeper
  • Wakes up often
  • Heavy breathing
  • Breathes through mouth in day
  • Hard to wake up in mornings
  • Sleepy in daytime
joey sleep evaluation
Joey: sleep evaluation
  • Evaluate other medications and AEDs
  • Pediatric sleep specialist
  • Polysomnogram:
    • No effect from EEG abnormalities on sleep issues
    • Significant number of obstructive sleep events
    • Recommend treatment with CPAP
joey autonomic function
Joey: autonomic function
  • Hands/feet cool, red, bluish for no reason?
  • Trouble regulating body temp? too hot, too cold?
  • Sweats too much? Doesn’t sweat enough?
  • Fast heart rate for no reason?
  • Fast breathing for no reason?
  • Flushing of the face/chest?
  • Pupils dilate?
joey autonomic
Joey: autonomic
  • Tolerate feedings ok?
  • Delayed gastric emptying?
  • Recurrent vomiting?
  • Diarrhea? Or constipation?
joey autonomic1
Joey: autonomic
  • “Always” has cold hands and feet
  • Trouble regulating body temperature
  • Always had trouble tolerating feedings—needs small volumes frequently
  • Constipated
joey food
Joey: food
  • On felbamate, best (only?) medicine for seizure control for 3 months
  • More GI upset, seems uncomfortable after feedings, doesn’t want to eat anything by mouth now
  • Has lost 3 lbs
joey s food medication side effect appetite loss
Joey’s food: MEDICATION SIDE EFFECT….appetite loss
  • Carbamazepine
  • Felbamate
  • Oxcarbazepine
  • Topiramate
  • Rufinamide
  • Valproate
  • Zonisamide
  • Most common AED “gut bombs”…most can do it for some!!
joey autonomic and food
Joey: autonomic and food
  • Recently had seen GI specialist “nothing wrong”
  • Discussed other options for seizure control
  • Trial of cyproheptadine initiated
joey motor
Joey: motor
  • Re-assess motor needs
  • Make sure Joey is being followed by rehabilitation medicine specialist or similar
  • Monitor for orthopedic problems that may emerge over time…scoliosis, etc
joey bone health
Joey: bone health
  • Assessment of risk:
    • Limited weight bearing, walking
    • On phenobarbital for 1 year in past
    • Premature infant
joey bone health1
Joey: bone health
  • Vitamin D level
  • Make sure getting vitamin D 800 IU/day
  • Calcium 1200 mg/day in divided doses
  • Maintain as much weight bearing as possible—stander, gait trainer
  • Needs long term monitoring for bone health
associated conditions
Associated conditions:
  • Cognition and learning:
    • now diagnosed with non verbal learning disability; adaptations in academics, home situation. Verbal IQ now known to be normal, planning altered for 1st grade.
    • Parents and teachers more aware of how his learning disabilities lead to frustration in multiple settings.
  • Sleep:
    • obstructive sleep apnea diagnosed. On CPAP, better sleep at night, more alert in day, less seizures overall
slide68
Autonomic function/GI
    • Able to tolerate feedings better with cyproheptadine; has gained 1 lb, no gagging
  • Motor:
    • referral to PM & R; new wheelchair and orthotics
  • Bone health:
    • on vit D and Ca supplements; monitor bone density over time
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