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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… Epilepsy

  • Identifying cause

  • Control of seizures


As time goes on
As time goes on: Epilepsy

  • Seizure control

  • Adverse effects of therapy

  • Associated conditions


Associated conditions are associated with
Associated conditions are associated with….? Epilepsy

  • 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: 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… Epilepsy

  • Brain injury from prematurity

  • Brain malformation

  • Infection

  • Stroke

  • Trauma

  • Metabolic disorder


Direct effects
“Direct effects” Epilepsy

  • 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: Epilepsy

  • 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… Epilepsy

  • 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 Epilepsy

  • 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)


The whole child conditions associated with pediatric epilepsy
Mood Epilepsy

  • Anxiety

  • Depression

  • Irritability

  • Mood swings


Behavior
Behavior Epilepsy

  • Oppositional

  • Irritable

  • Angry

  • Aggressive

  • Too passive, “not interested”

  • Hyperactive

  • Autism spectrum disorder


Sleep
Sleep Epilepsy

  • Excessive daytime sleepiness

  • Insomnia

  • Sleep disordered breathing

  • Delayed sleep phase

  • Behavioral

  • Seizure related sleep disruption


Autonomic function
Autonomic function Epilepsy

  • “Automatic” functions

    • Heart rate

    • Breathing rate/control

    • Blood pressure

    • Body temperature (core, extremities)

    • Sweating

    • Pupillary control

    • Gastrointestinal function

    • Bladder function


Food stuff
Food stuff… Epilepsy

  • Appetite

  • Weight gain or loss

  • Abdominal discomfort, nausea, vomiting

  • Feeding intolerance, delayed gastric emptying


Motor impairments
Motor impairments Epilepsy

  • Developmental coordination disorder

  • Low muscle tone (hypotonia)

  • Balance impairments

  • Cerebral palsy

    • Quadriplegia (all)

    • Hemiplegia (half)

    • Diplegia (both legs)


Bone health
Bone health: Epilepsy

  • 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: Epilepsy

  • 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: Epilepsy

  • 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 Epilepsy

  • 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)


The whole child conditions associated with pediatric epilepsy


Sally associated problems to screen for

Cognition Epilepsy

Mood

Behavior

Sleep

Autonomic

Food

Motor

Bone health

Other

Sally: associated problems to screen for…


Frontal lobe function
Frontal lobe function: Epilepsy

  • Emotional control center

  • Attention

  • Impulse control

  • Judgment


Frontal lobe function1
Frontal lobe function: Epilepsy

  • 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: Epilepsy

  • 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: Epilepsy

  • 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 Epilepsy

  • 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? Epilepsy

  • 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 Epilepsy

  • 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: Epilepsy

  • 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?


The whole child conditions associated with pediatric epilepsy


Sally s sleep
Sally’s sleep: Epilepsy

  • 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 Epilepsy

  • 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: Epilepsy

  • 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 Epilepsy

  • 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 Epilepsy

  • Obstructive sleep apnea

  • Anxiety/stress

  • Life style considerations

    • Family food habits

    • Exercise


Sally s motor
Sally’s Motor: Epilepsy

  • Always “a little clumsy”

  • Left handed

  • Slow, poor handwriting


Sally s motor1
Sally’s motor: Epilepsy

  • 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.


The whole child conditions associated with pediatric epilepsy

  • Sleep identification and management:

    • 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 identification and management:

  • 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


The whole child conditions associated with pediatric epilepsy
Joey: identification and management:

  • Born at 25 weeks gestation

  • Brain injury due to prematurity

  • Cerebral palsy; both legs (diplegia) and L arm/hand


The whole child conditions associated with pediatric epilepsy
Joey: identification and management:

  • 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 identification and management:

  • Cognition

  • Mood

  • Behavior

  • Sleep

  • Autonomic

  • Food

  • Motor

  • Bone health

  • Other


Joey cognition and learning
Joey: cognition and learning identification and management:

  • Has IEP in place

  • In Kindergarten

  • Mother thinks he is “smart”; has never been formally assessed


Joey cognition learning
Joey: cognition/learning identification and management:

  • 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 identification and management:

  • 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 identification and management:

  • 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 identification and management:

  • 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 identification and management:

  • 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 identification and management:

  • Tolerate feedings ok?

  • Delayed gastric emptying?

  • Recurrent vomiting?

  • Diarrhea? Or constipation?


Joey autonomic1
Joey: autonomic identification and management:

  • “Always” has cold hands and feet

  • Trouble regulating body temperature

  • Always had trouble tolerating feedings—needs small volumes frequently

  • Constipated


Joey food
Joey: food identification and management:

  • 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 identification and management:

  • 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 identification and management:

  • Recently had seen GI specialist “nothing wrong”

  • Discussed other options for seizure control

  • Trial of cyproheptadine initiated


Joey motor
Joey: motor identification and management:

  • 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 identification and management:

  • Assessment of risk:

    • Limited weight bearing, walking

    • On phenobarbital for 1 year in past

    • Premature infant


Joey bone health1
Joey: bone health identification and management:

  • 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: identification and management:

  • 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


The whole child conditions associated with pediatric epilepsy

  • Autonomic function/GI identification and management:

    • 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