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Developmental Disabilities and Sleep Disorders Not Recognized and Not Treated

Developmental Disabilities Nurses Association May 7 th Scott R. Stiefel, MD Sstiefel@hsc.utah.edu Division of Child and Adolescent Psychiatry Department of Psychiatry University of Utah. Developmental Disabilities and Sleep Disorders Not Recognized and Not Treated. Sleep is the Elixir of Life.

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Developmental Disabilities and Sleep Disorders Not Recognized and Not Treated

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  1. Developmental Disabilities NursesAssociationMay 7thScott R. Stiefel, MDSstiefel@hsc.utah.eduDivision of Child and Adolescent PsychiatryDepartment of PsychiatryUniversity of Utah

  2. Developmental Disabilities and SleepDisorders Not Recognized and Not Treated

  3. Sleep is the Elixir of Life

  4. SleepandBehavior

  5. Why is it important to always think about sleep disorders in this population?

  6. Most folks don’t tell us they are having problems - other than through their behaviors

  7. SLEEP DISORDERS • Almost completely ignored in this population • High incidence of central, obstructive, and mixed types of apnea in intellectual and developmental disabilities. • Sedation leads to irritability, inability to think and process and behavior problems – in all of us. • This is something that we can study, understand and treat. • Take the history • Recognize and treat the disorder

  8. When Should You Think about a Sleep Disorder? • Any one with severe behavior problems • Anyone who isn’t functioning as you would expect them to • Morning is their worst time • Severe snoring • Late afternoon fatigue and problems • Excessive daytime somnolence • Poor sleep at night • People on psychoactive medications, particularly polypharmacy

  9. Sleep Disorders in Genetic Syndromes • Downs Syndrome • Fragile X • Smith Magenis Syndrome • Prader Willi and Anglemans Syndromes • Cri du Chat • Tourette • Turners Syndrome • Craniofacial sequences and syndromes • Muscular Dystrophies • Connective Tissue Dysplasias and Marfan’s Syndrome • Rett’s Syndrome • Many others!

  10. Sleep Changes in Mental Illness • Depression • Bipolar Spectrum • Anxiety Disorders • PTSD • Psychotic Disorders • ADHD

  11. Chicken or the Egg or Both?

  12. Our Sleep Lab Experience

  13. Our ECT Experience

  14. Sleep 101

  15. Normal Sleep • The Electrocencephalogram • Beta Waves are seen with active mental concentration over the frontal lobes • Alpha Waves (8 to 12 cps) over the occipital and parietal lobes are seen when a awake person relaxes • Two major physiological states • Non-REM Sleep - Stages 1 through 4 • REM Sleep

  16. Non REM Sleep • 75 % of total sleep time • Stage 1 • 5% of total time • Low-voltage theta activity 3 to 7 cps • Stage 1 rapidly changes to stage two • Stage 2 • 45% of sleep time in young adults • Bruxism • Sleep spindles (12 to 14 cps) and slow high-amplitude groups of waves called K-complexes • Stage 3 and Stage 4 – slow wave sleep • 25% of sleep time in young adults • Mainly occurs during first half of sleep cycle • Deepest sleep, associated with sleepwalking (somnambulism), enuresis and night terrors • High-voltage delta waves at 0.5 to 2.5 cps

  17. REM Sleep • Rapid eye movements, decreased muscle tone low-amplitude, high-frequency brain waves (including alpha and beta) that form sawtooth waves • 25% of total sleep time in young adults • Occurs primarily during second half of sleep cycle • High degrees of brain activity and dreaming • Less likely to be woken by external stimuli but more likely to spontaneously awaken • Average REM latency is 90 minutes • Normal REM duration periods of 10 to 40 minutes • Suppression of sympathetic activity (miosis, penile and clitoral erection, body is essentially paralyzed • Historically it was thought that REM sleep and dreaming are important in learning and memory consolidation, this is not so clear at present – function of REM is unknown (Seigel 2001)

  18. Sleep Architecture • Sleep progresses through four stages of NREM which then occur in reverse order back to stage 1, followed by a period of REM. • Five to six cycle of NREM sleep with a REM period occur each night, each cycle taking approximately 90 minutes • Most psychoactive agents affect architecture • Neonates sleep 16 hours a day, 50% is REM • Young adults have REM percentage decreased to 25% • Disorders often missed when taking the history - common

  19. Sleep Deprivation • People deprived of sleep experience impaired physical and mental performance • If persists, they will demonstrate confusion, agitation, and ultimately psychosis and anxiety including paranoid delusions and hallucinations

  20. Neurochemistry of SleepSANDman • Serotonin and Acetylcholine (ACh) promote sleep • Increased availability of Serotonin increases total sleep time and delta wave sleep • Reduction in Serotonin decreases (for example destruction of the dorsal raphe nucleus • ACh in the reticular formation is associated with inducing REM sleep • In Alzheimer’s and normal aging, REM sleep, total sleep time and delta sleep are decreased

  21. Norepinephrine and Dopamine promote arousal and wakefulness • Increased levels of norepinephrine present in anxious patients may increase sleep latency and decrease total sleep time and the percentage of REM time • Increased levels of dopamine in mania and other psychotic illnesses are associated with decreased total sleep time

  22. Sleep Architecture with Aging • Further reduction in REM sleep • Decreased total sleep time • Decreased slow wave sleep • Increased nighttime awakenings

  23. Sleep Disorders • Dyssomnias • Problems with timing, quality, or amount of sleep • Insomnia, breathing-related sleep disorder, narcolepsy • Hypersomnias, circadian rhythm sleep disorder, restless leg syndrome, sleep drunkeness, and nocturnal myoclonus • Parasomnias • Abnormalities in physiology or in behavior associated with sleep, bruxism, nightmare disorder, sleep terrors, sleepwalking, and REM behavior disorder

  24. Insomnia • Difficulty falling asleep or staying asleep that occurs 3 times per week for at least 1 month and leads to sleepiness during day or impairs function • Seen in 30% of folks without DD/MR • Caffeine is a common cause • Stimulants, withdrawal from sedatives (ETOH, Benzos and opiates, etc.) • PAIN • Endocrine and metabolic disorders

  25. Insomnia continued • Stress • Depression • Anxiety • Physical Illness • Restless Legs Syndrome RLS • Caffeine • Irregular schedules • Circadian rhythm disorder • Drugs (alcohol, etc.)

  26. Insomnia in Mood Disorders • Some depressed patients often sleep excessively, but most have difficulty maintaining sleep • Depressed patients typically show normal sleep latencies • Manic patients (Bipolar Disorder) and anxious patients commonly have trouble falling asleep

  27. Treatment of Insomnia • Prescriptions with sedative agents are problematic and associated with changes in sleep architecture such as reduced REM and Delta sleep • These lead to problems with daytime sedation, dependence and tolerance and in the long run usually worsen things • Newer agents have fewer changes and are less likely to cause dependence and tolerance but are still problematic and do not improve slow wave sleep - zolpidem Ambien, Lunesta • Benzodiazepenes reduce likelihood of delta sleep and can treat night terrors and enuresis – but at a cost • Hygiene and ritual • Avoidance of caffeine • Exercise early in the day

  28. Sleep Apnea • Patients stop breathing for short periods of times (can be as many as 500 times) • Low oxygen and high CO2 • Daytime sleepiness • 10% of middle aged (not DD/MR) • Often related to depression, headaches, and pulmonary hypertension • Can be related to sudden death in infants and elderly and severe patients

  29. Sleep Apnea continued • Central Sleep Apnea • More common in elderly • More common in brain injury • Drive • Obstructive Sleep Apnea • Effort occurs but obstructs • Patient awakens • Stress • 8:1 males to females • Sometimes loud snoring • Pickwickian Syndrome • Daytime sleepiness from obstruction – obesity and body form

  30. Treatment of Sleep Apnea • In overweight patients – weight loss • Continuous Positive Airway Pressure CPAP • Uvuloplatoplasty • Tonsils and Adenoids • Last resort tracheostomy • Pacemakers with increased heart rate (Gottlieb 2002) ?

  31. Narcolepsy • Sleep attacks where fall asleep during daytime • Same amount of total nighttime sleep but have decreased sleep latency (less than 2 minutes), short REM latency (less than 10 minutes) reduced total REM, and interrupted REM (Sleep fragmentation) • 0.4 to 0.9% • Strong genetic component often seen first in adolescence • Hyponopompic (upon awakening) and hypnogogic (upon falling asleep) hallucinations • Cataplexy and sleep paralysis are seen in 30 to 70% of patients. • Cataplexy is loss of all muscle tone after a strong emotional stimulus • Sleep paralysis is the inability to be able to move for a few seconds after awakening • Eye muscles are active in both states as in REM

  32. Treatment of Narcolepsy • Prophylactically with stimulants and modafinil • Timed daytime naps • Antidepressants can be somewhat helpful if cataplexy is part of the clinical picture • High risk disorder

  33. What are the Functions of Sleep? • Sleep is of critical importance to the function of the cerebral cortex • Following sleep loss or deprivation, there is need for sleep recovery (Horne, 1988) • Most other parts of the body can recover during wakefulness

  34. Slow wave sleep demonstrates reduced metabolic rate • Increased metabolic rate is exhibited during REM sleep • If reduced metabolic rate indicates restoration than slow wave sleep is critical for restoration • (Maquet et al 1990, Horne 1988, 1992)

  35. Cerebral protein synthesis is enhanced in slow wave sleep • Growth hormone facilitates protein synthesis • Adrenaline and cortisol during wakefulness lead to cell breakdown and are reduced in sleep • Net effect – greater net protein synthesis in slow wave sleep • (Ramm and Smith 1990)

  36. REM is associated with early brain development and learning and memory • REM sleep increases after successful learning of an avoidance task • There appears to be a vulnerable period of time in which REM sleep must occur after learning to consolidate the learning. REM sleep windows (Smith and MacNeil 1992)

  37. Why do we sleep? • Jouvet in 1980 came up with an interesting theory that genetic programming of the brain occurs during REM sleep • In this model innate behaviors templates are reinforced during REM sleep • Helps to explain how aspects of heredity persist throughout life in an otherwise plastic nervous system • REM sleep would activate an endogenous system that could stimulate and stabilize receptors genetically programmed in some neuronal circuits

  38. Shibagaki, Kiyono, and Watanabe (1982) found delays in sleep spindles in Down Syndrome • Others have found differences in the amount of REM sleep in low and high functioning individuals with Down Syndrome • If certain kinds of learning do require REM sleep including tasks that involve the application of learned rules to solve novel problems – this is a big issue

  39. Evaluation of Sleep Disorders

  40. Ontogeny of Sleep • Time spent asleep • Distribution of sleep during a 24 hour period • Depth of sleep

  41. When safety is assured sleep lasts longer • What is the implication of this? • The organization of sleep over a 24 hour period changes dramatically over the life span • The alternation of sleep and waking state is called the circadian rhythm • Brainstem nuclei, the locus coeruleus and raphe nucleus and involved in generation of sleep stages • The suprachiasmatic nuclei are critical for the circadian rhythms of sleep and wakefulness

  42. Temperament • Children with “difficult” temperaments sleep less that children with “easy” temperaments (Weissbluth et al) • These children had lower sensory thresholds (Carey) • Keener et al, did not find difficult temperament of be associated with differences in sleep patterns

  43. Medical Problems Effecting Sleep • Pain • Arthritis • Osteoporosis • Heartburn • Cancer • Parkinson’s Disease • Dementia • Alzheimer’s Disease

  44. Medical Problems Effecting Sleep con. • Incontinence • Gastroesohageal Reflux • Incontinence • Nocturnal Cardiac Ischemia • Chronic obstructive pulmonary disease • Congestive Heart Failure • Peripheral vascular disease

  45. The Complete Exam • A detailed sleep history • General medical history • Complete review of symptoms • Complete mental health history • Complete social history • Psychological/developmental screening • Physical examination • Collateral history • Tools (video and audio recording, caregiver log,etc)

  46. Sleep History • Presenting complaint • Environment • Ritual • Onset • Duration • Character • Frequency

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