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Evaluation of the Sleepy Child: Objectives What to Do When the Sleep Study is Normal 1. Describe the appropriate clinical assessment of the sleepy child Stacey L. Ishman, MD • screening questions/ questionnaires Cincinnati Children’s Hospital Medical Center, • exam findings Cincinnati, OH • testing to screen for non-SDB causes of daytime sleepiness in children Thanks to: Scott Brietzke Susan Garetz Pell Wardrop 2

Objectives What We’ll Cover

2. Be familiar with the incidence, identification and impact of medical conditions that contribute to daytime tiredness including: 1) Evaluation of the sleepy child • narcolepsy • nocturnal seizures 2) Narcolepsy, RLS • restless leg syndrome 3) Circadian rhythm disorders • circadian rhythm disorders 4) Sleep hygiene

3. Recognize the importance of adequate sleep hygiene and review the components of good

sleep habits 3 4

Pediatric Sleep Disorders

Insomnia Sleep-related Breathing Disorders Hypersomnia (not related to breathing) Circadian Rhythm Disorder Parasomnia Sleep-Related Movement Disorders

Iglowstein I, et al. Pediatrics 2003;111:302–307

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Insomnia: Sleep Disorders Sleep Disorders – Chronic insomnia (3 months) Insomnia: Psychophysiological (Conditioned) Insomnia – Short-term insomnia (<3 months) – Learned sleep preventing associations – Normal Paradoxical insomnia Short sleeper (<6 hours) – Perception of severe sleep disturbance without Excessive time in bed evidence Idiopathic insomnia – Longstanding since childhood Inadequate Sleep Hygiene – ADLs inconsistent with good sleep Behavioral Insomnia of childhood – Limit-setting & Sleep-onset association

www.i.ehow.com www.i.ehow.com

Pediatric Sleep Disorders Pediatric Sleep Disorders

Sleep-related Breathing Disorders Sleep-related Breathing Disorders – Obstructive Sleep Apnea – Normal Variants Adult - Pediatric Snoring – Central Sleep apnea Catathrenia – sleep groaning Cheyne stokes – altitude – medical disorder Infancy – prematurity Treatment-emergement – Sleep related hypoventilation Obesity hypoventilation – congenital central alveolar hypoventilation syndrome – due med/dz – Sleep related hypoxemia Not covered in detail in this talk Not covered in detail in this talk

Pediatric Sleep Disorders Pediatric Sleep Disorders

Central disorders Hypersomnolence Circadian Rhythm Sleep-Wake Disorder: – Narcolepsy Type 1 - with cataplexy – Delayed Sleep-Wake Phase Syndrome – Narcolepsy Type 2 - without cataplexy – Advanced Sleep-Wake Phase Syndrome – Idiopathic Hypersomnia – Irregular sleep-wake rhythm disorder – Kleine-Levin Syndrome – Non-24 hour sleep-wake Rhythm disorder – Hypersomnia due to medical disorder – meds Free-running - psych – Shift work disorder – Insufficient sleep syndrome – Jet lag disorder – Circadian sleep-wake disorder NOS – Normal Variant Long sleeper

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Pediatric Sleep Disorders Pediatric Sleep Disorders Parasomnia: Parasomnia: –NREM-Related Parasomnias – REM-Related Parasomnias Disorders of Arousal Nightmare Disorder REM behavioral disorders Confusional Arousals Recurrent isolated sleep paralysis Sleepwalking – Other Parasomnias Sleep terrors Exploding head syndrome Sleep related eating disorder Sleep related hallucinations Sleep Enuresis Due to med disorder, medications, unspecified

Pediatric Sleep Disorders Pediatric Sleep Disorders

Sleep-Related Movement Disorders: Sleep-Related Movement Disorders: – Restless Leg Syndrome – Benign sleep myoclonus of infancy – Periodic Limb Movement Disorder – Propriospinal myoclonus of sleep onset – Sleep-Related Leg Cramps – Periodic Limb Movement Disorder – Sleep-Related Bruxism – Sleep-Related Movement Disorder due to – Sleep-Related Rhythmic Movement Disorder Med condition – medication/substance - unspecified Normal variants – Excessive fragmentary myoclonus – Hypnagogic foot tremor/alternating leg muscle activation – Sleep starts (hypnic jerks)

Pediatric Sleep Disorders: Infants Pediatric Sleep Disorders: School-Age Colic (1-4 months) – Melatonin metabolism? Sleep Hygiene Conditioned Insomnia Adjustment issues Adjustment Insomnia Anxiety related sleep issues Obstructive Sleep Apnea Parasomnia

www.futureforchildren.princeton.edu www.understanding-sleep-disorders.com

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Pediatric Sleep Disorders: Adolescents Sleep Hygiene!!! – Diet Caffeine Chocolate Anxiety Obstructive Sleep apnea Narcolepsy Klein-Levin Syndrome

www.azteenmagazine.com

Sleep Diary: For Please bring to clinic or fax to 410-955-0035, Attn: Dr. Stacey Ishman Remember to put your name/child's name on this form. Please make copies of this blankform. Evaluation of the Sleepy Child: History Instructions: Mark the time you/your child get in bed and are awake but trying to sleep, mark it with a down-arrow during that hour. l Mark the time you/your child is asleep by filling in the boxes during those hours. (see below) Medications Mark the time you/your child gets up (even if during the night). Use a(*) if woken up and an up arrow if waking up on your/their own(no alarm or being woken up by another)t. – Allergy meds (Diphenhydramine) Let me know if you/your child was sick or ifthere were unusual circumstances (a party, travel). Remember to log naps. – Antidepressants Evening/Night Morning Afternoon Date Day PM PM PM PM PM PM AM AM AM AM AM AM AM AM AM AM AM AM PM PM PM PM PM PM – ADHD medication timing 6 7 8 9 10 11 12 1 2 3 4 5 6 7 8 9 10 11 12 1 2 3 4 5 noon Mon Tues Wed Diet Thur Fri Sat – Caffeine Sun

Example below: – Chocolate / sugar Date Da PM PM PM PM PM PM AM AM AM AM AM AM AM AM AM AM PM PM PM 6 7 8 9 10 5 6 9 10 11 12noon 2 Mon * Comment: Could not fall asleep. I wokehim at 5am to go to doctor, he napped in the car later. Family History

Key: Asleep l In bed, awake t Out of bed, woke on their own * Out of bed, woken up by alarm etc. – Parasomnia – Insomnia/RLS

Evaluation of the Sleepy Child: Physical Exam Role of Sleep Study Height / Weight Usually not required for majority of sleep disorders BMI – Insomnia Observation of interaction with parent(s) – Straight-forward Parasomnia Sleep Apnea / Obstruction risk – Circadian Rhythm Disorders – Craniofacial structure Can be helpful in certain situations – Occlusion – Concerned about seizure – Neck Size – Movement disorders – Tonsil size / Mallampati Score May consider empiric treatment – Narcolepsy / Severe Hypersomnia Multiple Sleep Latency Test (MSLT) – Still concerned about OSA

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ICSD-2 Criteria for Narcolepsy Narcolepsy and Restless Leg • Characterized by excessive sleepiness • Two variants : Syndrome • Narcolepsy 1 (With Cataplexy) • Narcolepsy 2 (Without Cataplexy)

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Adapted from AASM 2005 Slide Set: Narcolepsy

Narcolepsy with Cataplexy (type 1) Symptoms of Narcolepsy Prevalence

• Excessive daytime sleepiness (EDS)

• Cataplexy Tetrad • Hypnagogic hallucinations Pentad • Sleep paralysis

• Fragmented nocturnal sleep

• Other associated features 37 1:4000 US 36

Adapted from AASM 2005 Slide Set: Narcolepsy – Data from Mignot 1998 Adapted from AASM 2005 Slide Set: Narcolepsy

Excessive Daytime Sleepiness Excessive Daytime Sleepiness

• Sleep attacks on a background of Wilkinson Addition Test Digit Symbol Substitution Test chronic sleepiness or fatigue • Frequent napping, usually refreshing • Memory lapses and automatic behaviors • Impaired attention / concentration

• Decreased work performance Control • Increased drowsy driving crashes Narcoleptic 3 Adapted from Mitler et al 1982 • Visual disturbances 38 9

Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

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Cataplexy Cataplexy • Muscle weakness triggered by emotions • Joking, laughter, excitement, anger • Brief duration, mostly bilateral • Sudden onset • May affect any voluntary muscle • Knee / leg buckling, jaw sagging, head drooping, postural collapse • Consciousness maintained at the start

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Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

Cataplexy Hallucinations • Vivid hallucinations at sleep onset (hypnagogic) or awakening (hypnopompic) • Auditory: sounds, music, someone talking to them • Visual: colored circles, parts of objects • Can be vividly realistic and anxiety provoking 42 43

Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

Sleep Paralysis Narcolepsy Age of Onset • Sudden inability to move on falling asleep or on awakening • Episodes are generally brief and benign, end spontaneously • Can cause significant anxiety • Associated with • Sleep deprivation • Narcolepsy

• Obstructive sleep apnea 44 45

Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

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Narcolepsy Environmental/ Narcolepsy Evaluation Developmental Factors • Most cases are sporadic • History • 1% to 2% 1st degree relatives have • Sleepiness, cataplexy, other disassociated narcolepsy-cataplexy REM sleep features • (RR= 20 - 40 times) • Polysomnography (PSG) • Exclude other causes for EDS (insufficient • Familial clustering in about 10% sleep, apnea) • Most monozygotic twins are discordant • Identify and treat associated conditions • Environmental & developmental factors • Multiple Sleep Latency Test (MSLT) are implicated • Objective sleepiness • Sleep onset REM periods (SOREMPs) 47 • CSF Hypocretin levels 48

Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

Narcolepsy Symptom Prevalence Narcolepsy Sleep Study Findings

• Short sleep latency • Sleep onset REM period in 50% of narcoleptics • Sleep fragmentation (REM and NREM) • Increased number of arousals • Increased stage 1 sleep • Low sleep efficiency • Frequently associated with periodic

limb movements 50 49

Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from AASM 2005 Slide Set: Narcolepsy

Narcolepsy Sleep Study Findings Narcolepsy Treatment

• The MSLT is: • Excessive sleepiness • A validated objective measure of the • Scheduled naps tendency to fall asleep • Indicated as part of the evaluation of • Modafinil & armodafinil patients with suspected narcolepsy • Sodium oxybate to confirm the diagnosis • Amphetamine (meth- and dextro-) • For 2 or more SOREMPs during MSLT: • Methylphenidate • Sensitivity was 0.79 • Selegiline

• Specificity was 0.98 51 52

From Arand et al 2005 Adapted from AASM 2005 Slide Set: Narcolepsy From Arand et al 2005 Adapted from AASM 2005 Slide Set: Narcolepsy

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Narcolepsy Treatment Seizure Differential

Table 1 Characteristics of Specific Sleep Disorders • Cataplexy, sleep paralysis, hallucinations and Seizures • Sodium oxybate Sleep Walk/Talk • Less evidence – cataplexy only Seizure Sleep Terrors RLS Narcolepsy • TCAs Incontinence + - - - • Amitriptyline, clomipramine Tongue biting + - - - Confusion + + - - • Fluoxetine Tonic-clonic movmnts + - - - Drooling + - - - Amnesia + + - - Occur while awake + - + +

PLMS, periodic limb movements of sleep; RLS, restless legs syndrome 53 55

From Arand et al 2005 Adapted from AASM 2005 Slide Set: Narcolepsy Adapted from Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.

Seizure Onset by Sleep Stage Seizures - History • Seizure onset primarily in 1/2 • Important to differentiate from parasomnias • Rare during ??? • Speech often stereotypical in seizures • Vs. sleep talking=speech is random • Occur predominantly in Stages 1/2 • Vs. sleep walking=usually in SWS • Atypical characteristics suggestive of seizure warrant full EEG evaluation • Nocturnal injury • Tongue or lip biting • Morning muscle soreness 57 56

Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293. Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.

RLS Diagnosis in Children RLS Diagnosis • What are the 4 Essential criteria • A compelling urge to move the limbs • Symptoms worse/only at rest • Variable/temporary relief by activity • Four essential adult criteria plus • Symptoms worse in the evening /night description in child’s own words consistent with leg discomfort OR • Four essential adult criteria plus two supportive criteria

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RLS Diagnosis in Children RLS Diagnosis • Supportive criteria • History • Sleep disturbance for age • Primarily a clinical diagnosis • Biological parent or sibling with RLS • 7 to 10% of population in US/ N Europe • Sleep study documenting periodic limb • Increases with age movement index ≥5/h • Strong FH correlates with earlier age of onset (<45yo) • Incidence • 2x more common in women • More common in whites than blacks • More common in iron deficient, pregnant 63 62 and end-stage renal disease

RLS Aggravators RLS Diagnosis • Nicotine • Supportive clinical features • Caffeine • In Children • Behavioral and mood difficulties • SSRIs • IncludingADHD • Metoclopramide • Cognitive issues • Prochlorperazine maleate • Sleep difficulties • Dopamine antagonists • Adults and children • Sleep disturbance • Diphenhydramine • Periodic leg movements • Alcohol • Response to dopaminergic therapy 64 • Family history 65 • Normal medical/physical evaluation

RLS Diagnosis RLS – Sleep Study • Rhythmic or semi-rhythmic movements of • Supplemental workup the legs • Polysomnography • Found in 90% of RLS patients during • Iron profile sleep • And/or neuropathy screen • > 5 periodic limb movements/hour in kids • > 15/hour in adults

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RLS – Iron Studies RLS Treatment • Behavioral techniques - *1st line in peds st • Iron deficiency defined as: • Sleep Hygiene - * 1 line in peds • Iron – very low or iron deficient • Ferritin level ≤ 40 ng/ml • Ferritin below 40 • acute-phase reactant • Dopaminergic agents • may misrepresent the true • Pramipexole - Rotigotine iron stores, so the transferrin • Ropinirole saturation determined along • Levodopa - augmentation with it • Transferrin saturation • Opiates – oxycodone, tramadol percentage ≤ 16% 68 • Antiepileptics – gabapentin, pregabalin69, carbemazepine

Incidence of Circadian Rhythm Circadian Rhythm Disorders NSF- 1/15 children in age range of 5- Disorders 13 years have CRD 200,000 children in US Adolescents reported prevalence up to 16% with Delayed Sleep Phase Syndrome

Circadian Rhythm Physiology of Circadian Rhythm

– Natural internal clock is slightly The suprachiasmatic nucleus longer than 24 hours (SCN)= pacemaker – Cues in our environment keep us Light is the strongest influence on on a 24 hour clock –light, meals, the sleep/wake cycle social activity Melatonin secretion signals sleep period

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Retino-hypothalamic Tract

Light information transmitted to the brain by non-visual neurons (ganglion cells) originating in the back of the eye

Types of Pediatric Circadian Advanced Sleep Phase Rhythm Disorders Syndrome

Delayed Sleep Phase Syndrome – Sleep and wake very early 6pm- 3am Advanced Sleep Phase Syndrome – May be genetic Irregular Sleep Wake Pattern – 1% Elderly Non-24 hours (Free-running) – Children- the early awakening is a problem for parents

Irregular Sleep Wake pattern Non-24 Sleep Wake pattern

– Random sleep wake pattern – History of insomnia or EDS or both – Lack of environmental cues- parental – > 3 months schedule – Sleep logs or actigraphy confirm (>14D) – Profound developmental delays – Blind 50% – Dementia

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Delayed Sleep Phase- Night General Treatment of CRDs Owls – Delay in sleep time by 2 or more hours Firm sleep/wake schedule- fixed – Difficulty arising in the morning AM arising time most important – Sleeping in very late on weekends Avoid weekend variation – Results in sleep deprivation during the week Sleep Hygiene- caffeine, computer, cell phones, lights, TV – Occurs in 7-16% of Teens

Melatonin Secretion Delay Factors contributing to DSPS

Evening BLUE light exposure - suppresses melatonin secretion – Computer screens – Video Games – Bright overhead lighting

History Treatment of DSPS Parents may not be aware of their teen’s sleep pattern Sleep Hygiene Teens may be reluctant to reveal their Firm Sleep Schedule sleep pattern Melatonin Chronotherapy

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Actigraphy - nl or not Actigraphy - nl or not

Actigraphy – nl or not Actigraphy – nl or not

Sleep Hygiene: General

Comfortable Sleep environment – Correct temperature Sleep Hygiene – Quiet – Dark

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Cheerios & Pampers Advice Cheerios & Pampers Advice

Age 1-2 years Pre-school age –Put child to –Establish a sleep when routine drowsy, but not already asleep

Sleep in Children Need to look at the bigger picture

“Mommy, can I have a glass of water ?”

Limit-Setting Sleep Disorder How many hours of sleep does

Behavioral insomnia of childhood the average teenager need? – Bedtime stalling and repeated demands Treatment Older school-aged children and teens – Consistency in routine and limit setting Day AND night time need on average about 9.25 hours of – Time warning(s) (bedtime in 30, 10, 5 minutes) sleep a day Carskadon MA et al. Sleep 1980 – Positive reinforcement Charts Rewards

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How many hours of sleep does Caffeine the average teenager get? 76% of children are getting caffeine from Sixth-graders sleep an average of 8.4 hrs on some source on a daily basis school nights Journal of the American Dietetics Association 2004 12th-graders sleep on average 6.9 hrs/ night 31% of teens consume at least 2 – 2 hours less sleep than recommended caffeinated beverages/ day Only 20 percent of teens get 9 hours of sleep Sleep in America Poll 2006 on school nights 31% of US teens drink energy drinks on a regular basis Sleep in America Poll 2006 Simmons research poll 2006

Caffeine Caffeine

Coca-Cola 12oz 34 mgcaffeine Caffeine takes up to 6 hours to be DietCoke 12oz 45 mgcaffeine excreted from the body RedBull 8.5oz 80 mgcaffeine Monster 19oz 160 mgcaffeine Teens who drink caffeinated beverages Cocaine energy drink 8.9 oz 280 mgcaffeine get less sleep than those who do not KIT KAT waferbar 1.5oz 6 mgcaffeine – Sleep in America poll 2006 HERSHEY'S KISSES 9 pieces 9 mg caffeine

Starbucks tall latte 12 oz 75 mg caffeine

School Performance School Performance 28 percent of high school students said they Eighty percent of students who get the fell asleep in class at least once a week recommended amount of sleep are 22 percent dozed off while doing homework achieving As and Bs in school, 14 percent of teens arrive late or miss school Student who get less sleep are more likely because they oversleep to get lower grades – Sleep in America poll 2006 Sleep In America Poll 2006

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Sleep and Mood Automobile Safety

Among teens who report being unhappy or Drowsy drivers cause about 100,000 accidents a tense year – 73% feel they don’t get enough sleep Over half of those crashes involve drivers from 16 to 25 years old – 59% report being too sleepy during day More than half of adolescent drivers (51 percent) Teens who sleep > 9 hours/night have driven while drowsy in the past year – Report more positive moods then peers 15% of drivers in grades 10-12 drive while Sleep in America poll 2006 drowsy at least once a week Sleep In America poll 2006

School Start Times School Start Times

Most middle and high schools start later Survey 10,000 6-12 grade students before than elementary schools and after school start time moved from 7:30-8:30 AM in Kentucky Teenagers often can’t fall asleep early, even if they are in bed The number of students who received at least eight hours of sleep per night increased from 35.7 % to 50 %. 16.5% drop in auto accident rates for teen drivers Journal of Clinical Sleep Medicine 2008

Recommendations Recommendations: Teens Parental Awareness Regular bedtime and sleep schedule Most teens know they're not getting Relaxing bedtime routine enough sleep – Half of teenagers admit they get less sleep – Reading, music than they need to be at their best Remove distractions from bedroom – 51 percent say they feel too tired or sleepy – TV, Phone, computer during the day – Use parental controls, check phone logs 90 percent of parents believe their adolescents are getting enough sleep on Avoid caffeine school nights – Especially after lunch – Sleep in America Poll 2006

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Parental Awareness: Summary Sleep Needs Sleep hygiene issues are by far the most common pediatric sleep problems

Sleepiness is not the most common way children with sleep disorders present

The sleep history and sleep diary are the most useful tools to make a diagnosis

The physical exam is of limited usefulness www.sleepfoundation.org PSG is only used in a minority of cases

Summary Summary • Narcolepsy can occur in children and is • Seizures must be differentiated from characterized by parasomnias • Excessive daytime sleepiness (EDS) • Can be increased with concomitant • Cataplexy (with = type 1, without = type 2) OSA • Hypnagogic hallucinations • Extended montage EEG with the • Sleep paralysis PSG may pick up nocturnal seizures • PSG with MSLT is the primary tool for • Tend to occur in NREM, rare in SWS diagnosis and REM • Full Neurologic evaluation with Extended EEG may be required

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Summary Summary • RLS is characterized by 4 essential • RLS in children – alternate criteria: features: • Four essential adult criteria plus two • An urge to move, usually associated supportive criteria with paresthesias • Supportive criteria • Onset or exacerbation of symptoms • Sleep disturbance for age (including daytime sleepiness) at rest • Biological parent or sibling with RLS • Relief of symptoms with movement • Sleep study documenting periodic limb • Symptoms manifesting in a circadian movement index ≥5/h pattern

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Summary Summary

Circadian Rhythm Disorders are common in Awareness of problem/ sleep needs of children children and adolescents Set a good example Even if SDB present, look for other sleep – Most adults are sleep deprived as well! disorders Consistency important In Teens- be especially aware of DSPS and Sleep conducive environment the possibility of sleep deprivation Control use of electronics and caffeine Elderly – consider advanced sleep phase Legislation Driving restrictions for teens Blind – consider non-24 (free running) School start times

Thank#8 You Stacey Ishman, MD, MPH Surgical Director, Upper Airway Center Multidisciplinary Treatment of OSA [email protected]

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