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Hypersomnolence of Central Origin

Hypersomnolence of Central Origin. Zafer Soultan, MD Director, Pediatric Pulmonary and Sleep Medicine Upstate, Golisano Children’s Hospital Syracuse, NY. Main sources. Classification of Sleep Disorders, AASM, third edition Sleep Medicine Pearls, Berry, R. third Edition. Sleepiness.

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Hypersomnolence of Central Origin

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  1. Hypersomnolence of Central Origin Zafer Soultan, MD Director, Pediatric Pulmonary and Sleep Medicine Upstate, Golisano Children’s Hospital Syracuse, NY

  2. Main sources • Classification of Sleep Disorders, AASM, third edition • Sleep Medicine Pearls, Berry, R. third Edition

  3. Sleepiness • Inability to stay awake during the major waking episodes of the day. • Large increase in total daily sleep without feeling of restoration • Sleepiness alleviated by naps, but reoccurs shortly • In children: • long night sleep, or reoccurrence of napping • Inattentiveness, emotional liability, hyperactivity and declined school performance • Must occur for at least 3 months

  4. Assessing severity; Epworth

  5. Assessing severity • Multiple Sleep Latency Test (MSLT): objective measure of sleepiness

  6. Before the test • Document adequate sleep for 1-2 weeks: sleep log and/or actigraphy • PSG a night before with 6-7 hours of sleep • Patients free of drugs that influence sleep for 14 days before study

  7. What do we measure • Mean sleep latency (MSL) • The number of sleep-onset rapid eye movement (REM) periods (SOREMPs)- within 15 minutes of sleep onset.

  8. MSLT: MSL > 8 minutes

  9. But, what about age variation Hence, consider MSL < 12 minutes abnormal in children

  10. Actigraphy

  11. Central Disorders of Hypersomnlence • Narcolepsy type 1 • Narcolepsy Type 2 • Idiopathic hypersomnia • Kleine-Levin syndrome • Insufficient sleep syndrome, the most common • Narcolepsy or hypersomnia due to medical condition • Hypersomnia due to medications or associated with psychiatric disorder

  12. Narcolepsy Type 1 (narcolepsy with cataplexy) • Daily periods of irrepressible need to sleep, or daytime lapses into sleep • One, or both of the following • Cataplexy anda mean sleep latency of ≤ 8 minutes and 2 or more SOREMP • CSF hypocretin -1 of ≤ 110 pg/ml or < 1/3 mean value of normal

  13. Sleepiness in narcolepsy • Daily episodes of irresistible need to lapse into sleep • Patient awaken refreshed • Likely to occur in monotonous situations that require no active participation • Physical activity may suppress the urge to sleep • Sleep attacks in unusual situations- eating or walking

  14. Cataplexy • Brief (< 2 minutes) of usually bilateral and symmetrical loss of muscle tone with retained consciousness • Precipitated by strong emotions- laughter, anticipation of an award (children) • In children: facial hypotonia (or generalized) with droopy eyelids, mouth opening, and protruded tongue, or gait unsteadiness

  15. Cataplexy

  16. Associated features • Disruption of nocturnal sleep • Hypnagogic hallucinations and/or sleep paralysis occur in 33-80% • Obesity • Depressive symptoms, anxiety (20%) • Sleep: REM sleep behavior disorder, OSA, PLMD, sleep talking • Fatigue

  17. Narcolepsy type 1 • Onset 10-25 years, and rare before 5-year age • Sleepiness and then cataplexy within 1-5 years. Rarely, cataplexy may precede sleepiness. • Usually stable when fully developed over the first few years, but cataplexy may decrease, or increase.

  18. Demographics/Etiology (genetics, HLA typing) • 1/4000 in western European. 7/4000 in Japan • Head trauma, viral illness, seasonal patterns (in the spring) • Autoimmune? • Increased in antibodies against beta-hemolytic streptococcus • In winter 2009-2010 after vaccination against H1N1 associated influenza (pandemrix), a dramatic spike in new cases of narcolepsy • Familial? all patients are positive for HLA DQB1*0602 • But, the risk of narcolepsy type 1 in first degree relatives is 1-2%, and only 30% chance in identical twin

  19. Children • Rare prior to age of 4 years • Sleepiness may be difficult to asses, or the child may exhibit hyperactive behavior and deterioration in school performance • Catplexy; weakness on the face, eyelids and mouth, tongue protrusion (cataplectic facies). • MSLT? • CSF Hypocretin-1 is highly sensitive and specific test

  20. Neurobiologic Mechanisms of Narcolepsy. Scammell TE. N Engl J Med 2015;373:2654-2662

  21. Hypocretin deficiency in human narcolepsy Nishino S. The Lancet 355(9197):39–40, 2000

  22. PSG • SORMP, very specific but not sensitive • REM sleep without atonia • Disruption of sleep with frequent awakenings • Increased N1

  23. Polysomnography in narcolepsy Meir H. Kryger, MD, Atlas of Clinical Sleep Medicine

  24. MSLT in narcolepsy Meir H. Kryger, MD, Atlas of Clinical Sleep Medicine

  25. Treatments

  26. Narcolepsy type II • Daily periods of irrepressible need to sleep, or daytime lapses into sleep • Cataplexy is absent • A mean sleep latency of ≤ 8 minutes, and 2 or more SOREMP (one SOREMP in the PSG a night before replaces one of the SOREMP on MSLT) • CSF hypocretin -1: > 110 pg/ml (immunoreactive assay) or > 1/3 of mean values of normal individuals obtained with the same assay

  27. Demographic/predisposing factors/complications and prognosis • 15-25% of the clinic narcoleptic patients • Low Hcrt-1 in 24%, 8% will have intermediate level. • 10% of patients will progress to type 1 • 45% of total narcolepsy type II will have HLA DQB1*0602 positive • The number of hypocretin producing cells are decreased • Triggers unknown: ?head trauma and viral illness • Starts in adolescence

  28. Children • Sleepiness may present with reappearance of regular napping • MSLT: no normative data in children < 6-year. • Adolescents: the most common etiologies of short sleep onset in MSLT, often with multiple SOREMPs, are chronic sleep deprivation and delayed sleep phase disorder • Behavioral problems may occur at the onset of the disorder. Symptoms may be hidden by the patients. • Inattentiveness, insomnia, lack of energy, bizarre hallucinations, or combination thereof can lead to psychiatric misdiagnosis

  29. Differential diagnosis • Idiopathic hypersomnia: • Similar sleep onset on MSLT, but < 2 SOREMP’s on MSLT/PSG • Long unrefreshing naps • High sleep efficiency and sleep drunkenness • OSA • Chronic sleep deprivation • Circadian Rhythm disorders; delayed phase and shift work • Malingering, and substance abuse, and medications • Chronic fatigue

  30. Narcolepsy 1 and 2 patients often are • Failing in school, and often dismissed from their jobs. • Driving may be avoided for fear of a motor vehicle accident. • The inability to sleep at night may further contribute to a loss of control these patients have over their schedule. • Depression and weight gain are common

  31. Medical Disorders causing Narcolepsy

  32. Idiopathic Hypersomnia (IH) • Daily periods of irrepressible need to sleep, or daytime lapses into sleep • No cataplexy • MSLT: < 2 SOREMP’s, or no SOREMP’s if the REM onset on PSG was ≤ 15 minutes • Mean sleep onset latency on MSLT ≤ 8 min, OR total 24-hour sleep ≥ 11 hours on PSG or actigraphy with sleep log • Insufficient sleep syndrome is ruled out

  33. Additional features • Sleep drunkenness • Long unrefreshing naps (>1 hour) • High sleep efficiency (>90%) on PSG • Autonomic system disturbance: headache, orthostatic disturbance, perception of temperature dysregulation, Raynaud phenomena, sleep paralysis and hypnogogic hallucination

  34. IH • Unknown pathophysiology • Onset adolescents 16-21 years of age • Remission in 14%

  35. Differential diagnosis • Narcolepsy II • OSA • Chronic fatigue • Hypersomnia with medical or psychiatric disorders, due to medications or substance • Long sleepers

  36. Kleine-Levin syndrome • At least 2 recurrent episodes of excessive sleepiness and sleep duration, each persisting for 2 days-5 weeks • Episodes recur more than once in 1 year, and at least once every 18 months • Normal alertness, mood, cognitive function and behavior between episodes

  37. Kleine-Levin syndrome • At least one of the following during the episode: • Cognitive dysfunction • Altered perception • Eating disorder • Disinhibited behavior (such as hypersexuality) • Sub-type: Menstrual-related Kleine-Levin Syndrome

  38. Kleine-Levin Syndrome • Incidence 1-2 per million • Male/Female: 2:1 • Starts in second decade of life and remits in 14 years

  39. Insufficient sleep syndrome • Daily periods of irrepressible need to sleep or daytime lapses into sleep or, in the case of prepubertal children, there is a complaint of behavioral abnormalities attributable to sleepiness • Sleep time is shorter than expected for age • The patient curtails sleep time • Extension of total sleep time results in resolution of the symptoms of sleepiness

  40. Associated features • Irritability, concentration and attention deficits, reduced vigilance, distractibility, reduced motivation, anergia, dysphoria, fatigue, restlessness, uncoordination and malaise • Common in adolescence • Diagnosed by 2-3 weeks of sleep diary and actigraphy • PSG; increased sleep efficiency. • MSLT excessive sleepiness, no SOREMP’s, and excessive N1

  41. Long Sleeper • > 10 hours in adults • 2 hours more than age-specific norms. • Sleepiness when they are forced to curtail their sleep time • It begins in childhood

  42. Case 1 • 17-year old male, EDS started at 9th grade • No cataplexy • No snoring

  43. Actigraphy

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