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Resources for Diagnosing Narcolepsy

Understand criteria for making a narcolepsy diagnosis and find ICD-10 codes for narcolepsy.

ICD-10 codes ICSD-3-TR criteria
ICD-10 codes » ICSD-3-TR criteria »

International Classification of Diseases, 10th Revision (ICD-10) Codes1

Narcolepsy With Cataplexy

G47.411

Narcolepsy Without Cataplexy

G47.419

International Classification of Sleep Disorders, 3rd Edition, Text Revision (ICSD-3-TR) Diagnostic Criteria2

Narcolepsy Type 1 (Narcolepsy With Cataplexy)

Criteria A-C must be met

  1. The patient has daily periods of irrepressible need to sleep or daytime lapses into drowsiness or sleep
  2. The presence of one or both of the following:
    1. Cataplexy (as defined under Essential Features in the full ICSD-3-TR diagnostic criteria for narcolepsy type 1)* and either:
      1. Mean sleep latency of ≤ 8 minutes and two or more SOREMPs on an MSLT performed in accordance with current recommended protocols†
      2. A SOREMP (within 15 minutes of sleep onset) on nocturnal polysomnogram
    2. CSF hypocretin-1 concentration, measured by radioimmunoassay, is either ≤ 110 pg/mL (using a Stanford reference sample) or < 1/3 of mean values obtained in normal subjects with the same standardized assay‡
  3. The symptoms and signs are not better explained by chronic insufficient sleep, a circadian rhythm sleep-wake disorder or other current sleep disorder, mental disorder, or medication/substance use or withdrawal

Narcolepsy Type 2 (Narcolepsy Without Cataplexy)

Criteria A-E must be met

  1. The patient has daily periods of irrepressible need to sleep or daytime lapses into drowsiness or sleep occurring for at least three months
  2. A mean sleep latency of ≤ 8 minutes and two or more SOREMPs on an MSLT performed in accordance with current recommended protocols.† A SOREMP (within 15 minutes of sleep onset) on the preceding nocturnal polysomnogram may replace one of the SOREMPs on the MSLT. §,
  3. Cataplexy is absent#
  4. If CSF hypocretin-1 concentration measured by radioimmunoassay is either > 110 pg/mL (when using a Stanford reference sample) or > 1/3 of mean values obtained in normal subjects with the same standardized assay**
  5. The symptoms and signs are not better explained by chronic insufficient sleep, a circadian rhythm sleep-wake disorder or other current sleep disorder, mental disorder, or medication/substance use or withdrawal

*Typical cataplexy is most strongly associated with narcolepsy type 1. Although some patients with narcolepsy type 1/hypocretin deficiency may present with atypical cataplexy features, presentations that include only atypical cataplexy should raise a higher index of doubt regarding a diagnosis of narcolepsy type 1. Clinical judgment is required. Further guidance regarding distinguishing typical from atypical cataplexy is included in the Essential Features section in the full ICSD-3-TR diagnostic criteria for narcolepsy type 1.

†See Krahn LE, Arand DL, Avidan AY, et al. Recommended protocols for the Multiple Sleep Latency Test and Maintenance of Wakefulness Test in adults: guidance from the American Academy of Sleep Medicine. J Clin Sleep Med. 2021;17(12):2489-2498. Sleep logs are required, accompanied by actigraphy, whenever possible, prior to in-laboratory sleep testing to evaluate for insufficient sleep and circadian rhythm disturbances.

‡If hypocretin deficiency is verified, the diagnosis of narcolepsy type 1 should be made regardless of other comorbidities that could potentially be related to clinical symptoms, given the definitive nature of this finding.

§The diagnostic value of a SOREMP on nocturnal polysomnography in the absence of MSLT SOREMPs is not established for narcolepsy type 2. However, it may help guide clinical decision-making, such as the need to pursue repeat or alternative testing.

Because the circadian clock strongly gates the propensity of REM sleep, narcolepsy type 2 should not be diagnosed in a shift worker without prior re-entrainment to a normal schedule.

#If cataplexy develops later, the disorder should be reclassified as narcolepsy type 1.

**If the CSF hypocretin concentration is tested at a later stage and found to be either ≤ 110 pg/mL (when using a Stanford reference sample) or < 1/3 of mean values obtained in normal subjects with the same standardized assay, the disorder should be reclassified as narcolepsy type 1.

CSF, cerebrospinal fluid; MSLT, Multiple Sleep Latency Test; SOREMP, sleep-onset REM period.

Diagnostic criteria from International Classification of Sleep Disorders, third edition, text revision. Copyright American Academy of Sleep Medicine. Reproduced with permission.

  1. ICD-10 Data. Sleep disorders G47-. Accessed October 5, 2023. https://www.icd10data.com/ICD10CM/Codes/G00-G99/G40-G47/G47-#G47.41
  2. American Academy of Sleep Medicine. International Classification of Sleep Disorders. 3rd ed. Text Revision. American Academy of Sleep Medicine; 2023.

Performance of routine tasks without awareness.

Sudden and brief loss of muscle tone, often triggered by strong emotions or certain situations. Narcolepsy with cataplexy is known as narcolepsy type 1.

Complete collapse to the ground; nearly all skeletal muscles are involved.

Only certain muscle groups are involved.

Biological clock mechanism that regulates the 24-hour cycle in the physiological processes of living beings. It is controlled in part by the SCN in the hypothalamus and is affected by the daily light-dark cycle.

Frequent awakenings and inappropriate transitions between states of sleep and wakefulness during nighttime sleep.

The inability to stay awake and alert during the day.

A neurotransmitter in the brain that supports wakefulness.

Vivid, realistic, and sometimes frightening dream-like events that occur when falling asleep.

Also known as orexin. A neuropeptide that supports wakefulness and helps suppress non-REM sleep and REM sleep.

Primary brain region for regulating the timing of sleep-wake states.

Unintentionally falling asleep due to excessive daytime sleepiness. Also known as “sleep attacks.”

Brief, unintentional lapses into sleep or loss of awareness.

A validated objective measure of the tendency to fall asleep in quiet situations.

People living with narcolepsy type 1 have low levels of hypocretin.

Narcolepsy without cataplexy; the cause of narcolepsy type 2 is unknown.

A state of sleep characterized by slower-frequency, more synchronized neuronal activity and decreased muscle tone. Deep stages help to restore the body.

A multiparameter test that monitors physiologic signals during sleep; used as a diagnostic tool in sleep medicine.

A state of sleep characterized by fast-frequency, desynchronized activity on EEG, vivid dreams, and loss of muscle tone. Normally occurs 60-90 minutes after sleep onset. Also known as “paradoxical sleep.”

Brief loss of control of voluntary muscles with retained awareness at sleep-wake transitions.

An abnormal sleep phenomenon characterized by REM sleep occurrence within 15 minutes of sleep onset; may occur during nighttime sleep or daytime napping.

A group of neurons located in the hypothalamus that are essential for promoting non-REM sleep. These neurons project to all wake-promoting regions to inhibit wakefulness and promote non-REM sleep during the night.

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US-NAR-2300169/Nov 2023

US-NAR-2300169/Nov 2023