Chair, Richard K. Bogan, MD, FCCP, FAASM, prepared useful Practice Aids pertaining to narcolepsy and idiopathic hypersomnia for this CME activity titled “Managing Disorders of Hypersomnolence: Reducing Patient Burdens, Protecting Patient Health.” For the full presentation, downloadable Practice Aids, and complete CME information, and to apply for credit, please visit us at https://bit.ly/3vX3ZTY. CME credit will be available until November 3, 2023.

1. Narcolepsy and Idiopathic Hypersomnia Diagnosis
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40
B. At least one of the following
• Cataplexy (a few episodes per month)
• Hypocretin deficiency in the absence of acute brain injury,
inflammation, or infection
• Nocturnal sleep polysomnography showing REM sleep latency
≤15 minutes, or a MSLT showing MSL ≤8 min and ≥2 SOREMPs
A. Recurrent periods of an irrepressible need to sleep, lapsing
into sleep, or napping within the same day occurring at least
three times per week over the past 3 months
DSM-5
E. Hypersomnolence and/or MSLT
findings not better explained by
other causes
D. CSF hypocretin-1 concentration
not measured or >110 pg/mL
or >1/3 of normal mean
C. Cataplexy is absent
B. At least one of
the following
• Cataplexy and
MSL ≤8 minutes
and ≥2 SOREMPs
on an MSLT
• CSF hypocretin-1
concentration
≤110 pg/mL or
<1/3 of normal
mean
B. MSL ≤8 minutes and ≥2
SOREMPs on an MSLT or a
PSG REM latency of
≤15 minutes as substituting for
one SOREMP on MSLT
A. Irrepressible need for sleep (≥3 months)
Type 1 Narcolepsya
(both A and B must be met)
Type 2 Narcolepsya
(A-E must be met)
ICSD-3
DIAGNOSTIC CRITERIA15-17
Mixed
• Cataplectic facies
Negative
• Transient loss of antigravity muscle tone that is frequently evoked by emotion
• Near continuous hypotonia without emotional stimulus
Active
• Hyperkinetic (compensatory) features that may be
enhanced by emotional stimuli
• Complex movement disorder
RECOGNIZING CATAPLEXY
(NARCOLEPSY TYPE 1)2,3,5,14
• Memory loss • Immune response
• Fatigue
• Apathy • Metabolic changes
• Accidents
• Automatic activity • Productivity impairment
• Microsleeps
• Autonomic tone changes
• Decreased alertness • Mood changes
SOME CONSEQUENCES OF NARCOLEPSY3,4,6,10-13
• Use of sedatives
• Shift-work sleep disorder
• Delayed sleep phase disorder
• Narcolepsy • Other medical conditions
(eg, hypothyroidism, Parkinson's
disease, Prader-Willi syndrome,
myotonic dystrophy)
• OSA • Depression
• Insufficient sleep • Idiopathic hypersomnia
DIFFERENTIAL DIAGNOSIS OF CHRONIC DAYTIME SLEEPINESS1,2
Narcolepsy Screening and Diagnosis
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Recognizing
and
Diagnosing
Narcolepsy4-10
Symptoms
Scales
Tests

2. Narcolepsy and Idiopathic Hypersomnia Diagnosis
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40
ICSD-3 Diagnostic Criteria: Idiopathic Hypersomnia
Daily periods of irrepressible need to sleep or daytime lapses into sleep, present for ≥3 mo
Absence of cataplexy
<2 SOREMPs on MSLT (or <1 if nocturnal REM latency ≤15 min)a
Presence of one or more of the following:
• MSL ≤8 min on MSLT
• Total 24-h sleep time ≥660 min on 24-h PSG or wrist actigraphy in association
with a sleep log (averaged over ≥7 d)
Insufficient sleep syndrome is ruled out
Hypersomnolence and/or MSLT findings are not better explained by other causes
Note that PSG 360 minutes is required prior to MSLT.
a
PSG SOREMP counts as one SOREMP added to MSLT.
1. https://www.sleepfoundation.org 2. Scammell TE. N Engl J Med. 2015;373:2654-2662. 3. Morse AM. Med Sci. 2019;7:106. 4. Scheer D et al. Sleep. 2019;42:zsz091. 5. Mirabile VS, Sharma S. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK549782/.
6. Thorpy M, Morse AM. Sleep Med Clin. 2017;12:61-71. 7. Antelmi E et al. Sleep Med Rev. 2020;50:101254. 8. https://rarediseases.org/rare-diseases/narcolepsy/. 9. Skjodt NM. Can Fam Physician. 2008;54:1408-1412.
10. https://narcolepsynetwork-org.s3.us-east-2.amazonaws.com/wp-content/uploads/2015/11/Narcolepsy-In-the-classroom.pdf. 11. https://narcolepsyireland.org/impacts-of-narcolepsy. 12. Barateau L et al. Sleep. 2019;42:zsz187.
13. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Narcolepsy-Fact-Sheet. 14. Szabo ST et al. Sleep Med Rev. 2019;43:23-36. 15. Ruoff C, Rye D. Curr Med Res Opin. 2016;32:1611-1622.
16. American Academy of Sleep Medicine. The International Classification of Sleep Disorders–Third Edition (ICSD-3). 3rd ed. Darien, IL: American Academy of Sleep Medicine; 2014.
17. American Psychiatric Association. Sleep-Wake Disorders; Narcolepsy. In: Diagnostic and Statistical Manual of Mental Disorders Fifth Edition (DSM-5). Arlington, VA: American Psychiatric Association; 2013:372-378.

3. Intervention
Strength of
Recommendation
Critical Outcomes Showing Clinically
Significant Improvementa
Excessive Daytime
Sleepiness
Cataplexy
Disease
Severity
Quality
of Life
Narcolepsy
Modafanil Strong   
Pitolisant Strong   
Sodium oxybate Strong   
Solriamfetol Strong   
Armodafinil Conditional  
Dextroamphetamine Conditional  
Methylphenidate Conditional  
Idiopathic insomnia
Modafanil Strong  
Clarithromycyin Conditional   
Methylphenidate Conditional 
Pitolisant Conditional 
Sodium oxybate Conditional 
Lower-sodium oxybate was approved by the FDA with an indication
for EDS and cataplexy of narcolepsy in 2020 and for IH in 2021;
that timing means it is not included in the AASM guidelines
Recommended Interventions for Narcolepsy
and Idiopathic Hypersomnia in Adult Populations
a
Accident risk and work/school performance/attendance were critical outcomes; however, no data were available.
 Critical outcomes showing clinically significant improvement.
American Academy of Sleep Medicine's Summary of Recommended
Interventions for Narcolepsy and Idiopathic Hypersomnia1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40

4. American Academy of Sleep Medicine's Summary of Recommended
Interventions for Narcolepsy and Idiopathic Hypersomnia1
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40
Strong recommendation:
“We recommend …”
The ultimate judgment regarding the suitability of any
specific recommendation must be made by the
clinician and the patient.
Conditional recommendation:
“We suggest …”
• Almost all patients should receive the recommended course of action
• Adherence to this recommendation could be used as a quality criterion
or performance indicator
• Most patients should receive the suggested course of action; however,
different choices may be appropriate for different patients
• The clinician must help each patient determine if the suggested course
of action is clinically appropriate and consistent with his/her values and
preferences
Implications of Strong and Conditional Recommendations
1. Maski K et al. J Clin Sleep Med. 2021;17:1881-1893.

5. Daily Symptoms in IH With or Without Long Sleep (≥10 Hours)
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40
Data From the Hypersomnia Foundation Registry
(N = 563 respondents)
Excessive daytime sleepiness
Intentional napping
Unintentional daytime sleep
Requiring multiple alarms to awaken
Having trouble waking up and functioning
with normal alertness
Brain fog (being unable to think clearly or
concentrate at any time throughout the day)
Difficulty remembering things
Automatic behaviors
222 (97.4%)
96 (42.1%)
74 (32.5%)
140 (61.7%)
158 (69.3%)
175 (78.1%)
156 (70.3%)
46 (21.6%)
IH Without Long Sleep
.70
< .0001
.10
.0002
< .0001
.01
.48
.58
P
235 (97.9%)
154 (64.2%)
95 (39.8%)
186 (77.5%)
211 (88.3%)
205 (86.9%)
170 (73.3%)
54 (23.8%)
IH With Long Sleep
1. Trotti LM et al. Sleep Med. 2020;75:343-349.

6. FDA “Voice of the Patient” Report on Narcolepsy
Bothersome
Symptoms
• Most bothersome symptom: EDS
– EDS includes “brain fog,” automatic behaviors
• Cataplexy, hallucinations, or sleep paralysis can be terrifying, especially
with unpredictable loss of control
• Complaints of insomnia, weight gain, mood fluctuations, and depression
Effects
Quality
of Life
• Narcolepsy limits employment opportunities
• Patients curtail social interactions and avoid activities or scenarios that might be
dangerous to themselves or others (eg, driving)
• Patients expressed frustration at “being labelled as lazy, careless, or incapable
by colleagues, healthcare professionals, and others”; accused of lying, faking
symptoms, or using illicit drugs
The FDA sought patient perspectives to better inform the drug development process,
with input from >120 patients or patient representatives
The Burdens of Narcolepsy and Idiopathic Hypersomnia1-4
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40

7. The Burdens of Narcolepsy and Idiopathic Hypersomnia1-4
Full abbreviations, accreditation, and disclosure information available at PeerView.com/JKX40
The Burdens of Patients With IH
Stigma
• Patients face stigma and are called lazy, inattentive, or unmotivated
Test Scores
• Patients with IH score lower than healthy controls on all domains of SF-36
scores (P = .009 to P < .01) except for physical function and bodily pain
Comorbidities
• Patients with IH are more likely to experience ANS, especially in orthostatic
and vasomotor domains
– More sleepiness and fatigue correlate with greater ANS burden, lower QOL
Survey Findings
• Patients with IH (N = 290) report struggling to maintain relationships
(65%), have suicidal thoughts (34%), and decide not to have children (25%)
1. https://www.fda.gov/media/88736/download. 2. Saini P, Rye DB. Sleep Med Clin. 2017;12:47-60. 3. Jennum P et al. Eur J Health Econ. 2014;15:303-311. 4. Whalen M et al. Sleep 2022.