Sleep and sleep disorders MUDr Katalin trbov Centrum

Sleep and sleep disorders MUDr. Katalin Štěrbová Centrum pro poruchy spánku u dětí Dětská neurologická klinika Fakultní nemocnice v Motole

Sleep physiology Examining sleep disturbances Sleep disorders

Sleep physiology n Sleep occurs periodically and is characterized by decreased reactivity to external stimuli ¨ decreased motions ¨ typical body position ¨ typical electrical activity of the brain ¨ n Sleep is immediately reversible n Sleep is an active process resulting from the cooperation of several regulatory centres n Wakefulness, NREM and REM sleep are three physiologic functional states

NREM sleep n n n Body resting, almost no movements Regular heartbeat and respiration, depression of blood pressure Almost no dreams Restorative function 3 stages: NREM I – drowsiness – eyelids closing, head-drop, voices grow away, thoughts dispersing, hypnagogic jerking ¨ NREM II – sleep spindles, K komplexes; easy to wake up ¨ NREM III – delta sleep, very regular heartbeat and respiration, hard to wake up ¨ n Muscles relaxed, no movements except sleepwalking

REM n n Irregular heartbeat and respiration, further depression of blood pressure Decreased thermoregulatory activity, no sweating, no shuddering REM sleep is very active compared to NREM: higher oxygen consumption, higher temperature of the brain, higher cerebral perfusion, EEG resembles wakefulness and drowsiness Muscles relaxed except extraocular and respiratory ones ¨ n n Muscle relaxation in neonates is not fully developed; newborns and small infants often jerk, vocalize, kick out, grimase Awakening somebody from REM might be difficult – outer and inner stimuli can be incorporated into dreams Dreams – their role is not very clear

NREM x REM n n The body is resting The mind is resting n The mind is active, but „disconnected“ from the body

Hypnogram NREM III REM 1% 45 -50% 25%

Development of sleep n n REM (active sleep) appears in the 6. -7. month of pregnancy NREM (quiet sleep) appears a month later In full-term neonates: 50% of sleep is „active“ sleep In preterm babies: 80% of sleep is „active“ sleep

Sleep requirements in children

Sleep regulation I. n Circadian clock in the ncl. suprachiasmaticus thalami control timing of sleep n Melatonin is released from the epiphysis in darkness and thus regulates the circadian clock in the hypothalamus

Sleep regulation II. n The „circadian clock“ regulates also other circadian rhythms as body temperature, level of cortisol, hunger n The inner „clock“ has to bee synchronized with the 24 hours cycle – according to light/darkness, food intake, social activities, external temperature and noise n Drowsiness and wakefulness varies during the day – drowsiness after lunch is normal, a period of increased alertness before bedtime is physiological n Owls and larks

Why do we sleep? n Both body and mind gets restoration during sleep n Different theories: mental and physical restoration, energy conservation, memory fixation, cool-down of emotions n Extracerebral processes: increased productin of growth hormone and thyreotropin, decreased salivation, decreased motility of bowels Immunity – long-term sleep deprivation has negative effect on immunity n n If somebody does not sleep one night, he is sleepy the other day and the only way to overcome sleepiness is to sleep

n Optimal length of sleep for an adult is 7 -8. 5 hours n After an acute sleep deprivation: NREM III and ½ of REM is compensated

Acute sleep deprivation Decreased efficiency n Decreased ability to learn n Instability of mood n Increased vulnerability of the – e. g. Increased risk of epileptic seizures n Worsened thermoregulation n Tremor, ptosis n

Chronic sleep deprivation n Trend of the last century in Western countries n Behaviourally induced insufficient sleep Increased day-time sleepiness Decreased efficiency Concentration affected Immune regulation deterioration Increased cardiac events Shorter life-expectancy Increased BMI n n n n

Sleep disorders

Sleep problems in the population n We spend about 1/3 of our life sleeping n Almost everybody experiences some sleep problem in his life n no systematic epidemiological studies

Diagnostic procedures n n n history EEG, sleep EEG, polysomnography, MSLT (Multiple Sleep Latency Test), MWT (Maintenance of Wakefulness Test), actigraphy ENT, paediatrics/internal medicine, gastroenterology, immunology Psychology/psychiatry Brain imaging HLA typization (95% of White patients with narcolepsy/kataplexy have the DQB 1*0602 haplotype)

Epworths sleepiness scale 0 = no chance of dozing 1 = slight chance of dozing 2 = moderate chance of dozing 3 = high chance of dozing SITUATION CHANCE OF DOZING Sitting and reading ______ Watching TV ______ Sitting inactive in a public place (e. g a theater or a meeting) ______ As a passenger in a car for an hour without a break ______ Lying down to rest in the afternoon when circumstances permit ______ Sitting and talking to someone ______ Sitting quietly after a lunch without alcohol ______ In a car, while stopped for a few minutes in traffic ______

Pediatric ¨ ¨ ¨ n n n n n Daytime Sleepiness Scale (PDSS) Scoring: 4 = Very often, Always 3 = Often, Frequently 2 = Sometimes 1 = Seldom 0 = Never Please answer the following questions as honestly as you can by circling one answer. 1. How often so you fall asleep or get drowsy during class periods? Always Frequently Sometimes Seldom Never 2. How often do you get sleepy or drowsy while doing your homework? Always Frequently Sometimes Seldom Never 3. * Are you usually alert most of the day? Always Frequently Sometimes Seldom Never 4. How often are you ever tired and grumpy during the day? Always Frequently Sometimes Seldom Never 5. How often do you have trouble getting out of bed in the morning? Always Frequently Sometimes Seldom Never 6. How often do you fall back to sleep after being awakened in the morning? Always Frequently Sometimes Seldom Never 7. How often do you need someone to awaken you in the morning? Always Frequently Sometimes Seldom Never 8. How often do you think that you need more sleep? Very Often Sometimes Seldom Never * Reverse score this item ¨ Abnormal Values: 6 th and 7 th Grade > 26, 8 th Grade >30

Wakefulness - PSG

PSG

International Classification of Sleep Disorders 1. Dyssomnias A. Intrinsic Sleep Disorders B. Extrinsic Sleep Disorders C. Circadian-Rhythm Sleep Disorders 2. Parasomnias A. Arousal disorders B. Sleep-Wake Transition Disorders C. Parasomnias Usually Asssociated with REM Sleep D. Other Parasomnias 3. Sleep Disorders Associated with Other Disorders A. Associated with Mental Disorders B. Associated with Neurologic Disorders C. Associated with Other Medical Problems 4. Proposed Sleep Disorders source: American Academy of Sleep Medicine, 2001

Insomnia I. n n Difficulty with falling asleep (sleep latency >30 min) Frequent arousals (sleep efficiency < 85%) Early wake up (30 minutes earlier than planned) Sleep has poor quality, non-refreshing, pat. has one on these complaints: ¨ Fatigue, concentration and memory deficit, mood disturbances, irritability, social discomfort, decrease of energy, motivation, propensity to errors, headache, insomnia anticipation

Insomnia II. n Acute insomnia (stress-related i. ) ¨ n Disturbed sleep is due to an acute stressor Primary (psychophysiologic, learned, conditioned) insomnia a disorder of somatized tension and learned sleep-preventing associations ¨ Individulas with P. I. typically react to stress with somatized tension and agitation. The meaning of stressfull events is denied and repressed but manifests itself as increased physiologic arousal (increased musce tension, increased vasoconstriction, . . ) ¨ Learned sleep-preventing associations ¨ n n exacerbate the state of high somatized tension and directly interfere with sleep consist mainly of marked overconcern with the inability to sleep; a vicious cycle then develops: patients in whom this internal factor (trying too hard to sleep) is a driving force for insomnia often find that they fall asleep easily when not trying to do so (e. g. Watching TV, driving, reading)

Insomnie III. n Paradoxical insomnia (sleep misperception) n Idiopathic insomnia (childhood onset i. , lifelong i. ) ¨ often with somnambulism, ADHD n Mental illness related insomnia

Insomnia IV. n Associated with neurological or other medical disorder n Associated with hypnotic-, alcohol- or stimulant dependence n Associated with inadequate sleep hygiene

Insomnia - therapy n Eliminating causes n Non – benzodiazepin hypnotics for shortterm (zolpidem) n Psychotherapy ¨ Cognitive-behavioral therapy

Sleep Hygiene Rules n n n n n Avoid drinking coffee, black or green tea, coke or energy drinks late afternoon (4 -6 hours before going to bed), reduce their consumption also during the day. Avoid eating heavy meals in the evening. Do not deal with problems that make you upset after dinner. Find some nice and calm activity to get rid of stress and get prepared for sleep. A short walk after dinner can improve your sleep. Avoid major physical activity 3 -4 hours before bed-time Do not drink alcohol to facilitate falling asleep – alcohol worsens the quality of your sleep Do not smoke before bedtime and during night-time awakenigs Use your bedroom and bed only for sleep and sex – remove TV set from your bedroom, do not eat and do not rest in your bed Go to bed and wake up at the same time every day (– + 15 minutes) Do not spend extra time in your bed lazing, thinking. Decrease noise and light in your bedroom to minimum; room temperature should be 18– 20 °C.

Insomnia of children n Sleep-onset association disorder Typically the child falls asleep under certain set of conditions (using a bottle, sucking on a pacifier, nursing, rocking) ¨ Return to sleep during night-time waking is difficult unless the conditions associated with sleep onset are re-established ¨ n Limit-setting sleep disorder The child refuses to go to bed at an appropriate time Asserts requirements verbally or leaving bed (drinking, eating, urination, more fairy- tales) ¨ „Curtain-calls“ ¨ ¨ n Medical reasons (pain, infant colic, itching) n Fear, anxiety

Sleep apnea Central sleep apnea syndrome n Obstructive sleep apnea syndrome n Central alveolar hypoventilation syndrome n

Obstructive sleep apnea syndrome

Normal breathing, obstructive hypopnea, obstructive apnea

OSAS n Video OSAS

OSAS in PSG recording

What is the problem with apnea? n Acute problem: each apnea/hypopnea is followed by desaturation and arousal → sleep fragmentation → bad quality of sleep → day-time symptoms (sleepiness, concentration problems) n Chronic consequences: arterial and pulmonary hypertension, obesity, increased risk of ischemic heart desease and cerebrovascular infarcts, decreased somatotropin release, insulin and leptin resistance

Therapy of OSAS Change diet and increase physical activity to decrease BMI n ENT surgery (adenotonsilectomy, plastic surgery on the soft palate) n Stomatosurgery n CPAP (continuous positive airway pressure) n

CPAP

Increased day-time sleepiness = decreased ability to maintain wakefulness during the day n Hypersomnia of central origin n ¨ Narkolepsy ¨ Recurrent hypersomnia ¨ Idiopathic hypersomnia n Hypersomnia due to other factors (organic brain disease; drugs, alcohol)

Narkolepsy n Symptoms: ¨ Excessive sleepiness with repeated episodes of naps or lapses into sleep of short duration ¨ Cataplexy (sudden loss of bilateral muscle tone propvoked by strong emotion) ¨ Sleep paralysis ¨ Hypnagogic hallucinations n n n PSG and MSLT: reduced sleep latency, sleeponset REM (SOREM) Genetic features (HLA typing: DQB 1*0602) Deficit of hypocretin (orexin) – peptid secreted in the hypothalamus

Idiopathic hypersomnia n Increased need of day-time sleep, but not episodic Recurrent hypersomia n Kleine-Levin syndrome ¨ Episodes of hypersomnia, hyperphagia, hypersexuality, mental status changes (aggression)

Therapy of hypersomnia n Changing day-time schedules n Medication ¨ Methylfenhydate ¨ Modafinil ¨ Sodiumoxybate ¨ Tricyclic antidepressants (imipramin), thymoleptics (cytalopram, sertralin)

Circadian-Rhythm Disorders I n Abnormal timing and length of sleep n Desynchronization of one’s biological rhytmicity and the external circadian rhythm ¨ e. g. non-24 hour sleep-wake disorder of blind

Circadian-Rhythm Disorders II. Delayed/advanced sleep-phase syndrome n Irregular sleep-wake pattern n Jet lag syndrome n ¨ Better tolerance of Western fligths n Shift work sleep disorder

Circadian-Rhythm Disorders III n Therapy: ¨ Regular physical activities and regular food intake to strengthen synchronization ¨ Morning illumination with bright light (2. 5 -10 tousand Lux) ¨ Melatonin ¨ Chronotherapy (extension of the day to 27 hours)

Parasomnias NREM x REM n NREM parasomnias – arousal disorders ¨ Confusional arousals ¨ Sleepwalking ¨ Sleep terrors n REM parasomnias ¨ REM sleep behavior disorders ¨ Nightmares – terrifying dreams provoke arousal with highly emotional and anxious reaction

Other parasomnias Bedwetting n Somniloqia (sleep talking) n Sleep-related eating n ¨ Compulsive ¨ Not provoked by hunger ¨ The patient eats inedible or toxic substances n Hypnagogic hallucinations

Abnormal movements related to sleep RLS n Bruxismus n Rhythmic movement disorder n

Restless Legs Syndrome n n n Disagreeable leg sensations that usually occur prior to sleep onset and cause an almost irresistible urge to move the legs Causes sleep onset insomnia Etiology ¨ Primary (idiopathic) ¨ Secondary (pregnancy, uraemia, anaemia)

Rhythmic movement disorder n video

Neurological disease related sleep disorders

Epilepsy and sleep I n n Sleep EEG recordings can show epileptic discharges that were not present in wakefulness In general epileptic discharges are more frequent in NREM then in REM sleep Sleep deprivation or bad quality sleep can provoke epileptic seizures Seizures appear typically during sleep or on awakening in some epilepsy syndromes

Neuromuscular disease n Sleep-related breathing disorder ¨ Decreased dilatation of the pharynx in sleep ¨ Inability to change position during sleep ¨ Decreased ventilation n Depression, anxiety

Cerebral palsy, neurodegenerative diseases n n n n n Limited perception of extrinsic stimuli Limited social contacts Limited abilities of education in mental retardation Altered ascendant reticular formation maintaining wakefulness Loss of circadian regulation Epileptic seizures Episodes of increased sleepiness, apathy, irritability Hyperactivity Hypnagogic jerking (sleep starts) interfering with falling asleep Pain, crying