The whole child conditions associated with pediatric epilepsy
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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


Things we think about at the beginning

Things we think about at the beginning:


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??


Associated conditions in kids with epilepsy

Associated conditions in kids with epilepsy…


Associated conditions in kids with epilepsy1

Associated conditions in kids with epilepsy:


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)


The whole child conditions associated with pediatric epilepsy

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)


The whole child conditions associated with pediatric epilepsy

  • 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?


The whole child conditions associated with pediatric epilepsy

  • 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.


The whole child conditions associated with pediatric epilepsy

  • 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


The whole child conditions associated with pediatric epilepsy

Joey:

  • 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:

  • 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


The whole child conditions associated with pediatric epilepsy

  • 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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