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Excessive Sleepiness By

Excessive Sleepiness By. Ahmad Younis Professor of Thoracic Medicine Mansoura Faculty of Medicine. Excessive Sleepiness.

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Excessive Sleepiness By

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  1. Excessive SleepinessBy Ahmad Younis Professor of Thoracic Medicine Mansoura Faculty of Medicine

  2. Excessive Sleepiness • Persons are considered excessively sleepy if they are unable to consistently achieve and sustain wakefulness and alertness to accomplish the tasks of daily living. Sleep occurs unintentionally or at inappropriate times or places. • In children, sleepiness may manifest as hyperactivity. • Excessive sleepiness can be defined using either clinicalor Multiple Sleep Latency Test (MSLT) criteria, • Hypersomnolence occurring almost daily for at least 3 months, and mean sleep latency of less than 8 minutes • It is important to distinguish excessive sleepiness from fatigue, exhaustion, tiredness, weariness, listlessness or weakness, which may closely mimic it.

  3. Demographics Excessive sleepiness is the most common complaint of patients presenting to sleep disorders centers.Excessive sleepiness is estimated to affect 5% of the general population. more prevalent among persons employed full-time compared with those who are employed part-time or unemployed.

  4. Severity of sleepiness Mild Sleepiness occurs during times of rest or when little attention is required (eg, reading or watching television)Sleepiness is associated with minor impairment of social and occupational functioning Moderate Sleepiness occurs daily and during mild physical activities that involve some degree of attention (eg, group meetings).Sleepiness is associated with moderate impairment of social or occupational functioning Severe Sleepiness occurs daily and during physical activities that involve mild to moderate degree of attention (eg, conversation, eating or driving). Sleepiness is associated with marked impairment of social or occupational functioning.

  5. SLEEPINESS BASED ON THE EPWORTH SLEEPINESS SCALE Severity of sleepiness Epworth score • Mild 10–12 • Moderate 13–17 • Severe > 17

  6. Consequences of Excessive Sleepiness • Greater risk of accidents (vehicular, industrial, or household) • Increased absenteeism, reduced work productivity, poor academic performance . • Mood disorder (depression or irritability) • Impaired interpersonal relationships.

  7. Common causes of excessive sleepiness 1- Inadequate sleep duration • Acute sleep deprivation • Chronic sleep deprivation • Insufficient sleep syndrome 2-Frequent awakenings and fragmented sleep • Obstructive sleep apnea syndrome • Upper airway resistance syndrome • Periodic limb movement disorder • Environmental sleep disorder

  8. Common causes of excessive sleepiness 3- Pathology of the central nervous system sleep-wake apparatus • Narcolepsy • Idiopathic hypersomnia • Post-traumatichypersomnia • Recurrenthypersomnia • Kleine-Levin syndrome • Menstrual-related hypersomnia 4- Disturbance of the endogenous circadian rhythm influencing the timing of wakefulness and sleep • Jet lag • Shift work sleep disorder • Delayed sleep-phase syndrome • Advanced sleep-phase syndrome • Non–24-hour sleep-phase disorder • Irregular sleep-wake pattern

  9. Common causes of excessive sleepiness 5- Drug or substance use • Administration of hypnotic and sedating medications • Withdrawal from stimulant agents • Adverse effects of medications 6- Other conditions • Medical disorders (CRF ,LCF ,Hypothyroidism) • Neurologic disorders (brain tumors, meningoencephalitis) • Psychiatric disorders (depression)

  10. Narcolepsy Definition • Narcolepsy is a neurologic disorder characterized by excessive sleepiness, and manifestations of REM sleep physiology during wakefulness (eg, cataplexy, sleep paralysis, and hypnagogic hallucinations). Demographics • Narcolepsy affects an estimated 0.05% of the general population often during adolescence or early adulthood (in the second decade of life), affect men slightly more frequently than women. • Excessive sleepiness is usually the presenting symptom, followed months to years later by cataplexy, sleep paralysis, and hypnagogic hallucinations. • Course is typically chronic, and symptoms persist lifelong.

  11. Pathophysiology • Hypocretin system. Loss of hypocretin (also known as orexin) neurons in the lateral hypothalamus appears to be the major underlying mechanism responsible for narcolepsy. • Hypocretins are neuropeptidesthat appear to have multiple functions, including regulation of sleep-wake cycle, appetite, body temperature, and blood pressure. • The hypocretin neurotransmitter system is located in the perifornical area of the hypothalamus, with wide projections to several wake promoting areas of the central nervous system (locus ceruleus, medullary reticular formation, raphe nuclei, and thalamus).

  12. Most wake circuits originate in the Brain stem arousal nuclei (BAN), which stimulate the thalamus, hypothalamus (Hyp) and basal forebrain.These projections also inhibit sleep centers

  13. The ventrolateralpreoptic nucleus (VLPO) in the hypothalamus inhibits the BAN and the parts of the hypothalamus involved in wakefulness. This leads to inhibition of other wake-centers including the thalamus, basal forebrain, and the cortex, and thus the initiation and maintenance of sleep.

  14. Neuroanatomy of NREM sleep • NREM sleepForebrain (anterior hypothalamus-preoptic region, includingventrolateralpreoptic area [VLPO] and basal forebrain) • Neurotransmitters serotonin and gammaaminobutyric acid (GABA). Other neurotransmitters include adenosine, norepinephrine,

  15. Neuroanatomy of REM sleep • REM sleepPons (pedunculopontinetegmental nuclei and the laterodorsaltegmental nuclei) Brainstem reticular formation, especially oral pontine reticular formation ,Other brainstem (lower medullary) and spinal cord neurons • Neurotransmitters: The main REM sleep neurotransmitter is acetylcholine. Other neurotransmitters include GABA and glycine.

  16. Pathophysiology • There are two types of hypocretin receptors (Hcrt), Hcrt-1 and 2. • Majority of patients with narcolepsy have decreased levels of Hcrt-1. • Low cerebrospinal fluid (CSF) levels of hypocretin in patients with narcolepsy with cataplexy have been described. • A defective cholinergic system regulating REM sleep (ie, muscarinicsupersensitivity) appears to contribute to the symptoms of narcolepsy.

  17. Genetics • There is a clear familial tendency in up to one-third of patients. • The risk of developing the disorder is increased by 10 to 40 times among first-degree relatives of narcoleptic individuals compared with the general population.

  18. Medical conditions causing narcolepsy Narcolepsy with cataplexy • Brainstem lesions (Degenerative ,Infectious ,Inflammatory , Neoplastic ) • Neoplastic (craniopharyngioma, gliomas, pituitary, and hypothalamic tumors) • Vascular (stroke or arteriovenous malformations) • Cerebellar ataxia • Hydrocephalus secondary to space-occupying lesions • Multiple sclerosis (hypothalamic) • Paraneoplastic syndrome (associated with anti-Ma2 antibodies) • Sarcoidosis (hypothalamic) • Viral illness (unspecified)

  19. Narcolepsy without cataplexy • Head trauma • Multiple sclerosis • Multiple system atrophy • Myotonic dystrophy • Parkinson disease

  20. Narcolepsy and sleep apnea • Myotonic dystrophy • Prader-Willi syndrome A Sleep onset REM periods and excessive sleepiness persist after adequate therapy of sleep apnea.

  21. Clinical Features of Narcolepsy Onset of narcolepsy is generally (between 15 and 25years of age). Excessive sleepiness is often the first symptom to appear, followed one to several years later by cataplexy, sleep paralysis, and sleep hallucinations. The classic clinical tetrad of narcolepsy consists of: • Excessive sleepiness • Cataplexy • Sleep paralysis • Sleep hallucinations Associated sleep disorders • Obstructive sleep apnea • REM sleep behavior disorder • Periodic limb movement disorder

  22. Clinical feature Prevalence 1-Excessive sleepiness. 100% 2-Cataplexy. 70%to80% 3- Hypnagogic Hypnopompic hallucinations. 8% to 70% 4- Sleep paralysis. 5% to 65%

  23. Excessive Sleepiness • Excessive sleepiness is chronic and may manifest as pervasive drowsiness and subwakefulness , frequent napping, microsleep episodes, and unexpected and overpowering sleep attacks occurring almost daily for at least 3 months. • Brief naps, lasting 10 to 20 minutes and seldom over an hour, occur repeatedly from 1 to 8 times throughout the day. Sleepiness is transiently relieved after awakening from a short nap only to gradually increase again within 2 to 3 hours. • Mechanism may be related to the loss of hypocretin neurons that stimulate arousal processes Sleep Attacks • A person may have sudden, irresistible periods of sleepiness, with sleep occurring during inappropriate places or circumstances. Sleep attacks can be preceded by a period of drowsiness, but they can also occur abruptly without warning.

  24. Cataplexy • Cataplexy is characterized by abrupt, transient, and bilateral loss or reduction of postural muscle tone occurring during wakefulness. • It is precipitated by intense emotion such as laughter, anger, fright, surprise, excitement, or embarrassment. • Rovery is generally immediate and complete, prolonged Status cataplecticusdescribes repetitive episodes of cataplexy occurring in succession that may last from several minutes to an hour; this may occur following abrupt withdrawal of REM sleep suppressants. Episodes of cataplexy may give rise to REM sleep with hypnagogic hallucinations and dreaming. • Cataplexy generally first develops several months or years after the onset of excessive sleepiness, but may, occasionally, be the presenting complaint of patients with narcolepsy

  25. Episodes of muscular atonia or hypotonia vary in: • Duration (lasting from a few seconds to several minutes) • Progression (severity being maximal at the start of the attack or worsening over several seconds or minutes) • Severity (ranging from mild weakness, such as drooping of the eyelids or sagging of the jaw, to complete lack of postural tone with a collapse to a chair or the ground) • Frequency (from once or twice yearly to as often as several times each day) • Body area affected (regionally affecting the face, neck, and extremities, or entire body) • Respiratory and oculomotor muscles are spared, and memory and consciousness are unaffected.

  26. Mechanism • Cataplexy can be considered an intrusion of REM sleep-related muscle atonia during wakefulness. Polysomnography Demonstrates wakefulness during brief attacks and REM sleep during more prolonged attacks. • Cataplexy is the only pathognomonic symptom of narcolepsy, because normal individuals may occasionally experience sleep paralysis and/or hypnagogic hallucinations. • The absence of cataplexy does not exclude a diagnosis of narcolepsy.

  27. Differential diagnosis • Syncope • Orthostatic hypotension • Transient ischemic attack • Vestibular dysfunction • Epilepsy (partial complex, atonic or absence seizures), • Neuromuscular weakness, • Psychosis, conversion disorder, or malingering.

  28. Sleep Paralysis • Transient loss of the ability to move occurring at sleep onset (hypnagogic) or upon awakening (hypnopompic). • It occurs in approximately 25% to 80% of persons with narcolepsy. • Less frequently, it can be seen either in an isolated form or in normal persons during sleep deprivation. Recurrent sleep paralysis can affect about 4% of the normal population. • Sleep paralysis involves all voluntary muscles with sparing of the respiratory and ocular muscles; lasts from several seconds to a few minutes; and is frequently accompanied by hypnagogichallucinations, dyspnea, and a sensation of dread. • Sensorium is generally unaffected. • Recovery, either spontaneously or following external stimulation (being touched or spoken to), is immediate and complete. • It often develops several months to years following the onset of excessive sleepiness.

  29. Differential diagnosis • Normal persons during sleep deprivation • Transient (hyperkalemic or hypokalemic) paralysis. • Isolated and familial (transmitted as an X-linked dominant trait) sleep paralysis

  30. Sleep Hallucinations • They are not pathognomonic for narcolepsy, and have been described in normal persons as well. • Recurrent sleep hallucinations can be seen in about 4% of the normal population. • Hallucinations may occur during wakefulness at sleep onset (hypnagogic) or on awakening (hypnopompic). • Hallucinatory phenomena often last a few seconds or minutes and can be visual (seeing a stranger or object in the room), auditory (being spoken to), tactile (a touch or a sensation of warmth or cold) or kinetic (a sensation of movement). • Often the experience has a fearful quality such as being attacked or escaping from danger, and this can be accompanied by sleep paralysis. • Sleep hallucinations often begin several months to years after the onset of excessive sleepiness.

  31. Clinical subtypes of narcolepsy 1- Narcolepsy with cataplexy with normal hypocretin-1 levels inCSF of cases Normal CSF hypocretin-1 levels are present in 10% 2-Narcolepsy without cataplexy with low hypocretin-1 levels Low CSF hypocretin-1 levels (< 110 pg/mL) are present in the CSF up to 10% to 20% 3-Narcolepsy with cataplexy-like or atypical episodes Low CSF hypocretin-1 levels are present in up to 20% 3- Isolated cataplexy Rare familial cases with early onset 4- Hypocretin gene mutations Early-onset (6 months of age) narcolepsy with cataplexy

  32. Narcolepsy Secondary to Medical Disorders (referred to as secondary narcolepsy). • Diagnosis requires documentation of narcolepsy either clinically (eg, presence of chronic excessive sleepiness for at least 3 months, or cataplexy) or objectively (MSLT demonstrating short mean sleep latency less than 8 minutes and at least 2 sleep onset REM periods) and a coexisting medical condition that is responsible for sleepiness. • Sleep may either be normal or moderately disrupted during polysomnography. • CSF levels of hypocretin-1 are low (< 110pg/ml or 30%of normal control values).

  33. Narcolepsy without Cataplexy • This form of narcolepsy is not associated with cataplexy; however, cataplexy-like symptoms may be described, including prolonged episodes of tiredness or muscle weakness related to atypical triggers (exercise, stress, or sex). • It accounts for about 10% to 50% of cases of narcolepsy. • Most patients have normal levels of CSF hypocretin-1. • Some cases of narcolepsy without cataplexy are associated with loss of hypocretin-containing hypothalamic neurons (but to a lesser degree than that seen in narcolepsy with cataplexy).

  34. Polysomnography • Decreased Sleep latency • Decreased Total sleep time • Increased Frequency of arousals • Increased Body movements • Decreased NREM stages 3 and 4 sleep • Decreased REM sleep latency (in about 50% of cases) Associated conditions: • Obstructive sleep apnea • Central sleep apnea • REM sleep behavior disorder • Periodic limb movements of sleep

  35. Multiple sleep latency test • Decreased Sleep latency • Two or more sleep onset REM NB:Narcolepsy with cataplexy can be diagnosed by clinical history alone. A thorough evaluation of medication and substance use as well as sleep, medical, neurologic and psychiatric history is mandatory. • Polysomnography followed by MSLT is indicated when cataplexy is absent, atypical or equivocal.

  36. Maintenance of Wakefulness Test • The Maintenance of Wakefulness Test (MWT) measures a person’s ability to remain awake in quiet situations. • It might be useful for monitoring treatment response to stimulant medications used for excessive sleepiness. Cerebrospinal Fluid Hypocretin-1 • CSF hypocretin-1 level < or equal to 110 pg/ml (or < one-third of mean normal control values) is highly specific and sensitive for narcolepsy with cataplexy but is less commonly present in cases without cataplexy. • A normal test does not exclude the diagnosis of narcolepsy with cataplexy (CSF hypocretin-1 levels are normal in up to 10% of cases). HLA Typing • Narcolepsy is associated with certain human leukocyte antigens (HLA), namely DR2 (particularly the subtype DR15) and DQ1 (in particular DQ6 [DQB1*0602]).

  37. ICSD-2 diagnostic criteria of Narcolepsy with cataplexy: • EDS daily for >3 months • Definite history of cataplexy - sudden and transient episodes of loss of motor tone triggered by emotions • Diagnosis of narcolepsy should, whenever possible, be confirmed by PSG followed by MSLT, the latter showing sleep latency </= 8 minutes and >/= 2 SOREMs. Alternatively, hypocretin cerebrospinal fluid levels </=110 picograms/mL • Hypersomnia is not better explained by another sleep, neurological, mental, or medical condition, medicine or substance use.

  38. ICSD-2 diagnostic criteria of Narcolepsy without cataplexy: • EDS daily for >3 months • Typical cataplexy is not present • Diagnosis of narcolepsy MUST be confirmed by PSG followed by MSLT, the latter showing: sleep latency </= 8 minutes and >/= 2 SOREMs • Hypersomnia is not better explained by another sleep, neurological, mental or medical condition, medicine or substance use.

  39. ICSD-2 diagnostic criteria of narcolepsy due to medical condition • EDS daily for >3 months • One of the following is present:1. A definite history of cataplexy, defined as sudden and transient episodes of loss of muscle tone (muscle weakness) triggered by emotion, is present.2. If cataplexy is not present or is very atypical, PSG followed by MSLT, the latter showing: sleep latency </= 8 minutes and >/= 2 SOREMs despite sufficient nocturnal sleep prior to the test (minimum 6 h).3. Hypocretin-1 levels in the CSF are <110 pg/ml (or 30% of normal control values), provided the patient is not comatose. • C. A significant underlying medical or neurological disorder accounts for the daytime sleepiness. • D. The hypersomnia is not better explained by another sleep disorder, mental disorder, medication use, or substance use disorder.

  40. Differential diagnosis of sleepiness • Idiopathic hypersomnia • Insufficient sleep syndrome • Inadequate sleep hygiene • Circadian rhythm sleep disorders • Medication use, abuse or withdrawal • Obstructive sleep apnea • Periodic limb movement disorder • Recurrent hypersomnia

  41. Differential diagnosis of cataplexy Transient ischemic attacks Vestibular disorders Akinetic seizures Hypotension Conversion disorder (pseudocataplexy) Sleep paralysis Neuromuscular disorders Malingering

  42. Pharmacologic therapy of narcolepsy 1- Excessive sleepiness and sleep attacks • Dextroamphetamine • Methamphetamine • Methylphenidate • Modafinil 2- Cataplexy, sleep paralysis and sleep hallucinations • Carbamazepine • Clomipramine • Fluoxetine • Imipramine • Nortriptyline • Protriptyline 3- Sleep disruption • Hypnotic agents • Sodium oxybate

  43. Dosing of stimulant medications • Modafinil100 to 400 mg/day • Dextroamphetamine 5 to 60 mg/day in 2 to 3 divided doses • Methylphenidate 5 to 15 mg 2 to 3 times a day

  44. Behavioral therapy for narcolepsy • Avoidance of sleep deprivation • Maintenance of regular sleep-wake schedules, and avoidance of shifts in circadian sleep-wake rhythms (eg, shift work) • Avoidance of prolonged inactivity during the daytime • Regular schedule of daytime naps (=15 minutes in duration) • Appropriately timed use of physical activities and caffeinated beverages to improve alertness and maintain wakefulness • Maintenance of optimum weight • Avoidance of stress

  45. Idiopathic Hypersomnia Definition sleepiness occurs after sufficient or even increased amounts of nighttime sleep and without any identifiable cause. Clinical Features • Excessive sleepiness is generally severe and constant. • Sleepiness, manifesting as normal or extended major sleep episodes (often lasting over 8 hours with few or no awakenings) and naps (up to 1 to 2 hours of NREM sleep) ,typically unrefreshing, protracted periods of daytime drowsiness, and paroxysmal sleep attacks, is present almost daily for at least 3 months. • It is associated with impairment of daytime function. • Cataplexy is distinctively absent

  46. Types of idiopathic hypersomnia 1- Idiopathic hypersomnia with long sleep time: • Prolonged nocturnal sleep duration (at least 10 hours) • At least one daytime nap lasting more than 1 hour. 2- Idiopathic hypersomnia without long sleep time: • Nocturnal sleep of normal or slightly prolonged duration (greater time than 6 hours but less than 10 hours.)  Demographics • Idiopathic hypersomnia accounts for about 1% to 10% of patients referred to sleep clinics for excessive sleepiness. usually begins insidiously during adolescence or early adulthood (onset often before 25 years of age). • Course is typically chronic. • Some cases of idiopathic hypersomniamay be familial with an autosomal dominant inheritance pattern

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