5/28/2020 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 1 5/28/2020 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 2 5/28/2020 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 3 5/28/2020 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 4 5/28/2020 ICSD-2 Criteria for Narcolepsy Narcolepsy and Restless Leg • Characterized by excessive sleepiness • Two variants : Syndrome • Narcolepsy 1 (With Cataplexy) • Narcolepsy 2 (Without Cataplexy) 34 35 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 5 5/28/2020 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 40 41 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 6 5/28/2020 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