5. Hypersomnia and EDS
Hypersomnia (嗜睡症) is characterized by excessive
daytime sleepiness (EDS)(白天) and/or
prolonged nighttime sleep(晚上)
EDS (日間嗜睡): fall asleep repeatedly during the day,
often at inappropriate times (at work or during a meal)
5
6. 6
Excessive daytime sleepiness
Increased need of sleep
睡眠需求增加
Insufficient sleep
睡不夠
Fragmented sleep
睡不好
Insomnia 失眠
Circadian rhythm
sleep disorders
日夜節律睡眠障礙
Insufficient
Sleep syndrome
睡眠不足
Drug-related
Insomnia
Sleep related
movement disorders
睡眠相關動作障礙
Narcolepsy
猝睡症
Obstructive sleep
apnea
睡眠呼吸中止症
Hypersomnia and EDS
8. Hypersomnia
Diagnosis Distinguishing Characteristics
Narcolepsy
Cataplexy, hypnagogic hallucinations, sleep
paralysis
Obstructive sleep apnea
Snoring, witnessed of apnea, large tongue,
obesity
Other medical disorders Hypothyroidism, Parkinson’s disease
Insufficient sleep
Sleepiness decreases with more sleep on
weekends and holidays
8
13. Assessment - Questionnaire
Pittsburgh Sleep Quality Index - PSQI
Cutoff:
PSQI > 5 yielded a diagnostic sensitivity of 89.6%
and specificity of 86.5% for poor sleeper
Each of the sleep components: 0 – 3
PSQI total score: 0 - 21
The higher total score indicating “worse sleep quality”
13
14. Assessment - Actigraphy
A non-invasive method of monitoring human
rest/activity cycles.
Useful for determining sleep patterns and circadian
rhythms
Objectively measuring sleep and average motor activity
over a period of days to weeks
腕動計
14
15. Assessment - PSG
In-lab standard polysomnography (PSG) is the
gold standard of Sleep study
EEG EOG
Air flow
EMG
ECG
Tho. & Abd. movement
SpO2
多項睡眠生理檢查
15
17. 17
Total sleep time 睡眠總時間
Sleep efficiency 睡眠效率
Sleep latency, REM latency 睡眠潛時, 快速動眼潛時
Percentage of NREM and REM period 睡眠分期%
Assessment - PSG
Arousal index 覺醒指數
Apnea-hypopnea index 呼吸中止指數
Oxygen desaturation index 低血氧指數
Periodic limb movement index 週期性肢體抖動指數
多項睡眠生理檢查
18. Assessment - MSLT
Multiple sleep latency test - MSLT
Record sleep latency and sleep onset REM periods
Five 20-minute nap opportunities set 2 hours apart
Participants must remain awake for the entirety of the
2 hours between nap
多次入睡時間測定
18
20. Background
The arousal spectrum is linked to the actions
of 5 main neurotransmitters
Norepinephrine (NE)
Acetylcholine (ACh)
Serotonin (5-HT)
Dopamine (DA)
Histamine (HA)
20
23. Orexin system
Orexin-A and orexin-B (食慾激素) are neuropeptides
synthesized in the lateral hypothalamus and
perifornical area
Orexin neurons receive signals related to environmental,
physiological and emotional stimuli, and project broadly
to the entire CNS
23
24. Orexin system
Orexin neuron stimulate histaminergic neurons,
noradrenergic neurons, serotonergic neurons and
cholinergic neurons
→ increase arousal state and activate the cortex
HA
NA
5-HT
Ach
24
Ach
Orexin
25. Orexin system
Orexin deficiency results in narcolepsy in humans
Orexin system is particularly important for
maintenance of wakefulness
Orexin system stabilized sleep architecture and
NREM-REM transition
25
26. Narcolepsy
Narcolepsy is a chronic neurologic disorder
affecting approximately 1 in 2500 people
The age of onset ranges from early childhood
to the fifth decade (10 – 50 years old)
26
Estimate Narcolepsy in Taiwan: 6000 – 11000 people !
29. The sleepiness is present everyday although can
be better on some days
Sleepiness is most evident when the patient is
sedentary or inactive
(watching TV, reading, or sitting quietly)
Narcolepsy – Daytime sleepiness
The patient is never free of sleepiness !
(ESS > 17)
29
30. Narcolepsy - Cataplexy
Cataplexy is characterized by sudden, emotionally
triggered episodes of muscle weakness with
preserved consciousness (意識清楚!)
30
33. Vivid dreams (鮮明的夢境) at sleep onset are a
common feature and often can occur prior to
falling asleep leading to hypnagogic hallucinations.
(usually visual but can be auditory)
Narcolepsy - Hallucinations 33
34. Sleep paralysis leading to an inability to move
for seconds or minutes
Narcolepsy – Sleep paralysis
Occurring in the transition from sleep to wakefulness
or from wakefulness to sleep, often in association
with dreams or hallucinations
34
鬼壓床 !
35. Narcolepsy - Diagnosis
When narcolepsy is suspected, all patients should
undergo both an overnight Polysomnogram (PSG)
and a daytime Multiple Sleep Latency Test (MSLT)
35
多次入睡時間測定
多項睡眠生理檢查
Day 2 09:00 – 17:30
Day 1 21:00 – 07:00
36. Narcolepsy - Diagnosis
PSG may show the features in narcolepsy of reduced
sleep efficiency, short sleep latency, and/or a short
REM latency
PSG will rule out other causes of sleepiness such as
sleep apnea or periodic limb movements of sleep
36
PSG should demonstrate an adequate amount of
sleep (≥ 6 hrs), including REM sleep
37. Narcolepsy - Diagnosis
The MSLT, performed the next day, will show a
reduced mean sleep latency of ≤ 8 min and
two or more sleep onset REM periods (SOREMPs)
during the 4 or 5 nap test.
37
睡眠潛時縮短 ( 平均 SL ≤ 8 min )
入睡出現快速眼動睡眠 (2次以上)
38. Narcolepsy - classification
Type 1 narcolepsy is caused by extensive loss of
hypothalamic neurons that produce the
neuropeptides “orexin”
Type 2 includes most of the same symptoms, but its
cause is unknown
38
41. Treatment
Most people with narcolepsy require treatment with
wakefulness-promoting medications
Modafinil (Provigil) is the first-line choice for EDS
→ an acceptable side-effect profile and has a
low potential for abuse
41
Pilositant (Wakix) is the first and only EMA and FDA-
approved non-scheduled treatment for EDS or
cataplexy in narcolepsy
42. Antidepressants reduce REM sleep and substantially
reduce cataplexy
Venlafaxine generally has an acceptable side-
effect profile, and it is effective for most of the day
Clomipramine potently suppresses cataplexy and
can be quite helpful when taken before an event
that is likely to trigger cataplexy
42
Treatment
43. Treatment
Medication Indication Mechanism Dose
Modafinil/
Armodafinil
EDS DA re-uptake inhibitor
200-400 mg QD AM
Armodafinil: 150-250 mg AM
Pitolisant EDS/cataplexy
HA receptor reverse
agonist
8.9 – 35.6 mg QD AM
Solriamfetal EDS
DA and NE re-uptake
inhibitor
75 – 150 mg QD AM
Methylphenidate EDS
DA, NE, 5HT re-uptake
inhibitor
5 mg BID, max: 60mg/d
Amphetamine/
Dextroamphetamine
EDS
DA, NE, 5HT re-uptake
inhibitor
10 mg QD, max: 60 mg/d
Oxybates EDS/cataplexy GABA? 2.25-4.5 g HS, max: 9 g HS
43
46. Summary
Narcolepsy is characterized of excessive daytime
sleepiness, Cataplexy, sleep paralysis, and
hypnagogic hallucinations
When narcolepsy is suspected
→ refer to medical center with Sleep Lab for
Overnight Polysomnogram (PSG) +
Multiple Sleep Latency Test (MSLT)
46