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1
Sleep & Developmental Disabilities:
Lessons for All Children
Lawrence W. Brown, MD
Pediatric Neuropsychiatry Program
Sleep Disorders Center
The Children’s Hospital of Philadelphia
March 28, 2012
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2 Importance of Sleep
Intrinsic scientific interest
Reflection of brain development
Significant behavioral consequences
Relevance to neurological disorders
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3 Sleep - Definition
A reversible behavioral state of perceptual
disengagement from and unresponsiveness
to the environment
Unlike coma, a physiologic, recurrent, and
reversible condition
A complex amalgam of physiological and
behavioral processes
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4 How do doctors study sleep?
Polysomnography
Multiple Sleep Latency Test
Actigraphy
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5 Polysomnography
Sleep staging (EEG, eye
leads, muscle leads)
Airflow (nasal & oral)
EKG
Chest and abdominal wall
motion
End-tidal CO2
Oxygen saturation
Video
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6 Multiple Sleep Latency Test
Semi-quantitative test of daytime sleepiness
4-5 nap opportunities throughout the day
– Patients asked to try to fall asleep in bed in
darkened room every 2 hrs
– 20 min opportunity to fall asleep; 15 minutes of
recorded sleep
Norms available above 6 yrs old
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7 Actigraphy
Accelerometer worn on wrist that measures
body movement
Correlates to wakefulness and sleep (including
non-REM vs REM)
Device can be worn for weeks; much more
accurate than sleep diary
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8 Idealized Sleep Histogram
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9 Idealized Sleep Histogram
Note stage 3-4 non-REM sleep
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10 Idealized Sleep Histogram
Note REM sleep
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11 Normal Sleep in Infants
Term infant - 16-18 hrs sleep
– 3-4 hr cycle throughout day
– Increasing day wakefulness and
night sleep by 1 month
6 month– mean sleep 14.2 hrs
– 6-8 hours of continuous night
sleep most common
6-9 months - increased
nocturnal awakenings
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12 Normal Sleep in Toddlers
Gradual decline in total sleep time
– From mean of 13.9 hrs at 1 yr to 11.4 hrs at 5 yrs
1-2 naps/day totaling 2-4 hrs
– Frequent short naps or long late afternoon nap may
interfere with night sleep
– Most children give up daytime nap by age 3
Sleep problems in 20-40%
“Good sleepers” awaken as often as “poor
sleepers”
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13 Normal Sleep in Older Children
Decline in total sleep time– From mean of 11.1 hrs at 5 yrs
to 10.2 hrs at 9 yrs
Typical “ideal” sleep/wake schedule– No difficulty in falling asleep
– Least likely to need alarm clock
– Optimal daytime alertness (mean MSLT=19 min)
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14 Normal Sleep in Adolescents
Further decline in total sleep
– From mean of 9.0 hrs at 13 yrs to
7.9 hrs at 16 yrs
Sleep architecture maturation
– 40% decline in SWS; slightly more
stage 2 NREM; stable REM
Increased daytime sleepiness
– Present even if total sleep stable
– Tendency to delayed sleep and
waking
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15 Common Pediatric Sleep Problems
Infant– Poor consolidation of
sleep-wake cycle
– Difficulty settling
Toddler– Behavioral disorders
– Non-REM parasomnias
– Obstructive sleep apnea
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16 Behavioral Sleep Disorders in Early
Childhood
Sleep-onset associations
Unstructured or inconsistent routine
Highly stimulating bedtime activities
Night fears, anxiety
Acute illness
Activating medications
Neurological disabilities
*Settling problems: 20% of 1-3 yr olds and 10% of 4 yr olds
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17 Graded Approach to Sleep Resistance
Common sense– Consistent sleep schedule
– Regular bedtime routine
– Avoidance of overly stimulating activity
Must first distinguish behavioral sleep disorder from medical conditions such as apnea, reflux
Structured behavioral interventions from graduated extinction (“Ferberizing”) to limiting bedtime hours
Medication only as last resort
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18 Non-REM Parasomnias
Sleep walking, sleep talking, agitated
arousals, sleep terrors
Typically first 1/3 of night (1-4 hrs)
Worse with sleep deprivation, stress
Difficulty in arousing child from event
Little or no recall of event
Must be distinguished from seizures
Family history in 60%
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19 Treating Non-REM Parasomnias
Reassurance
Environment safeguards
Scheduled awakenings– Only evidenced-based non-drug treatment
– Awaken child 15-30 minutes before expected event
– Effective in 50%
Low dose benzodiazepines– Clonazepam, diazepam
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20 Other Common Pediatric Parasomnias
Rhythmic movement disorder
– Head banging, body rocking
REM sleep disorders (nightmares)
– Most often in last 1/3 of night
– Occur in >50% of 5-7 yr olds
Enuresis
– 15% at 6 yrs with 15% resolution per yr
(which still leaves 2% at 13 yrs)
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21 Apnea: Definition
Apnea: absence of airflow at the nose/mouth
Obstructive apnea: No airflow despite respiratory
effort (due to airway obstruction)
Central apnea: No airflow or respiratory effort
(often due to CNS dysfunction)
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22 Obstructive Sleep Apnea: Nocturnal Signs
Noisy respirations and loud snoring
Observed apnea, gasping, choking
Restless sleep
Night sweats
Enuresis
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23 Obstructive Sleep Apnea: Daytime Signs
Morning headache, dry mouth
Overactivity
Attention problems
Learning difficulties
Irritability
Aggression
Fatigue
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24 Treating Obstructive Sleep Apnea
Tonsillectomy & Adenoidectomy
Continuous Positive Airway Pressure
Weight loss
Other surgery– Palatal reconstruction
– Craniofacial repair
– Tracheostomy
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25 Common Pediatric Sleep Problems
Older Child
– Insomnia
– Obstructive sleep apnea
– Sleep schedule disorders
– Daytime sleepiness
– Periodic limb movements of sleep
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26 Periodic Limb Movements of Sleep and
Restless Legs Syndrome
PLMS: Brief, repeated jerks of extremities
during sleep every 0.5-5 sec, cycle every 5-90
seconds
Often seen with Restless Legs Syndrome in
adults, but children rarely complain
– Desire to move extremities, usually associated with
discomfort and motor restlessness
– Relief by movement
– Increased in evening and during periods of rest/
inactivity
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27 Evaluating PLMS in Children
Usually presents as insomnia or “growing
pains”
Family history - positive in >70%
Serum ferritin as indication of iron depletion
Consider renal failure, diabetes, thyroid
function studies, B12, folate, EMG/ Nerve
conduction studies
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28 Treating PLMS and RLS
Avoid drugs that may aggravate symptoms
– Antihistamines, neuroleptics, SSRIs
Iron therapy for low ferritin (<50 mcg/L) or iron
saturation < 16%
Medications
– Dopaminergic agents (pramipexole, ropinirole)
– Other drugs (clonazepam, clonidine, gabapentin)
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29 Common Pediatric Sleep Problems
Adolescent
– Insomnia
– Daytime sleepiness including
inadequate sleep, delayed sleep phase
syndrome, narcolepsy
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30 Insomnia in Adolescents
Inadequate sleep time and poor sleep hygiene
– Late bedtime, irregular sleep schedule, availability
of multiple electronic devices, caffeine
– All compounded by increasing autonomy
Natural tendency toward delayed sleep
– Delayed melatonin release
Consider substance abuse
Primary insomnia and other sleep disorders
are unusual but need to be ruled out
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31 Treating Adolescent Insomnia
Screen patients for concurrent use of non-
prescription remedies, alcohol, drug, pregnancy
Rule out primary sleep disorders
Emphasize sleep hygiene
– Avoid caffeine, excessive exercise, bright light
Medications for short term use only
– No refills without reassessing target symptoms and
evaluating patient compliance
Choose medication with appropriate duration
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32 Selected Drugs for Pediatric Insomnia
Melatonin 1-10 mg– Sleep promoter taken 30-45 min before bedtime
Clonidine – 0.025-0.3 mg – Short action < 4 hrs
Zolpidem 5-10 mg
– 6-8 hour effect; rare residual drowsiness
Mirtazapine 15-45 mg
Trazadone 25-100 mg
Diphenhydramine 25-50 mg
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33 Selected Drugs for Pediatric Insomnia
Melatonin 1-10 mg– Sleep promoter; taken 30-45 min before bedtime
Clonidine – 0.025-0.3 mg – Short action < 4 hrs
Zolpidem 5-10 mg – 6-8 hour effect; rare residual drowsiness
Mirtazapine 15-45 mg
Trazadone 25-100 mg
Diphenhydramine 25-50 mg– Little evidence for chronic usage, side effects include
paradoxical activation, increased seizures
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34 Delayed Sleep Phase Syndrome
Inability to sleep at socially
appropriate time
“Night owl”
No objective sleep abnormality
– Can sleep in and awaken refreshed if allowed to
extend time in bed
Consider secondary gain
– Unusual schedule avoids both parental control of
night activities and school attendance
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35 Treating Delayed Sleep Phase
Syndrome in Adolescents
Melatonin 3-10 mg 30-45 min before lights out
Chronotherapy– Gradually advance bedtime by 10-15 min
or delay bedtime by 2-4 hours per night until desired effect achieved
– Importance of strict adherence to schedule once entrained
Light therapy– AM light resets biological clock
Medication as last resort
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36 Sleep and Developmental Disorders
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37 Sleep and Epilepsy
60% of children with epilepsy have seizures while asleep
Secondarily generalized nocturnal seizures
Benign rolandic epilepsy– Facial twitching, speech arrest, drooling, secondary
generalization
Nocturnal frontal lobe epilepsy– Bizarre clinical manifestations
(thrashing, laughter, agitation, bicycling movements)
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38 Sleep and Epilepsy
Drowsiness and non-REM sleep facilitate
epileptic discharges
Seizures can disrupt sleep; post-ictal lethargy can
disrupt sleep-wake schedule
Drugs can lead to sleepiness or insomnia
Disturbed sleep can lead to cognitive-behavioral
deterioration
Children with epilepsy not immune to inadequate
sleep and/or primary sleep disorders
*Bottom line: better sleep may improve seizure control
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39 Epilepsy and Sleep Apnea
Apnea associated with increased seizures– Almost 1/3 of patients with intractable epilepsy have
sleep apnea
– Treatment of apnea improves seizure control
AEDs causing weight gain (valproate, carbamazepine, gabapentin) may induce or worsen apnea
Sedating AEDs (phenobarbital, clonazepam) produce upper airway relaxation and reduce arousability
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40 Sleep and Selected Genetic Syndromes
Down syndrome
(obstructive apnea)
– Obesity, mid-facial and
mandibular hypoplasia,
marcoglossia, adeno-
tonsillar enlargement
Smith-Magenis syndrome
(severe insomnia)
– Sleep disorders in >75%
– Abnormal melatonin
production
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41 Sleep and Autism
Severe sleep problems independent of cognitive level
Most sleep disorders are behavioral– Inappropriate sleep associations
– Stereotypies - headbanging, rocking
– Excessive anxiety, rituals
– Communication problems
Must consider nocturnal seizures and epileptic autistic regression
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42 Sleep and ADHD
Frequent settling problems,
restless sleep, night
arousals, early arousals or
difficulty awakening
Primary sleep disorders
rarely cause ADHD, but may
exacerbate symptoms
– Obstructive sleep apnea
– PLMS
– Sleep phase disorders
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43 Sleep and ADHD
Co-morbid conditions may contribute to sleep problems– Depression
– Anxiety disorders
– Migraine
Medication issues– Stimulants may decrease sleep need
– Rebound hyperactivity if stimulants wear off too early
Parenting factors– Child allowed to set own schedule to avoid tantrums
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44 Sleep and Tourette Syndrome
Sleep onset difficulties, restless sleep, early awakening
PSG may show sleep fragmentation or persistence of tics in all sleep stages
Increased incidence of parasomnias and migraine
Sleep abnormalities increased with co-morbid ADHD and anxiety/OCD
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45 Recent Sleep Research in Tourette
Syndrome
80% of unselected university based clinic patients age 7-17 had > 1 sleep related problem – sleep onset insomnia
– poor sleep efficiency
– frequent arousals
– parasomnias
– nightmares
20% had > 4 sleep related problems
Sleep problems linked to reduced quality of life
Anxiety linked to increased problems
Storch et al, 2009
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46 Back to the Basics: When to Suspect
Underlying Sleep Disorder
Delayed sleep onset
Prolonged or frequent night awakenings
Restless sleep
Snoring, apnea
Decreased total sleep time
ADHD, irritability, aggression
Excessive daytime sleepiness
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47 Summary: Treating Sleep Disorders in
Developmental Disabilities
Always identify and treat underlying medical
condition
– Epilepsy
– Cardiorespiratory problems including apnea,
hypoventilation
– Pain – muscle spasms, contractures
– Medication effects – stimulants, sedatives, AEDs,
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48 Summary: Treating Sleep Disorders in All
Children
Sleep hygiene
– Consistent bedtime and regular sleep routine
– Consistent morning awakening
– Maintain daytime wakefulness
– Allow appropriate naps
Non-pharmacologic treatment
– Chronotherapy
– Light therapy
Sleep-promoting drugs, only if necessary
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49 Summary: Treating Sleep Disorders in All
Children
Pharmacotherapy
Melatonin 1-10 mg 30-45 min before sleep
Clonidine 0.05-0.3 mg
Mirtazipine 15-45 mg
Trazadone 25-100 mg
Intermittent benzodiazepines acceptable
Avoid diphenhydramine, if possible
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50 Selected References:
Meltzer LJ et al. Sleep and sleep disorders in children and
adolescents. Psychiatric Clinics of North America. 29: 1059-1076, 2006.
Mindell JA et al. Pharmacologic management of insomnia in children and
adolescents: consensus statement. Pediatrics 117: e1223-1232, 2006.
Kotagal S. Parasomnias in childhood. Sleep Medicine Reviews 13: 157-
168. 2009.
Koh S et al. Sleep apnea treatment improves seizure control in children
with neurodevelopmental disorders. Pediatric Neurology 22: 36-39,
2000.
Storch EA et al. Sleep-related problems in youth with Tourette’s
syndrome and chronic tic disorder. Child and Adolescent Mental Health
14: 97-103, 2009
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