Diagnostic Criteria

ICSD-3 Diagnostic Criteria

The American Academy of Sleep Medicine (AASM) International Classification of Sleep Disorders – Third Edition (ICSD-3), released in February 2014, contains diagnostic criteria for sleep disorders, including narcolepsy. The ICSD-3 features content changes from ICSD-2, including new nomenclature, classifications, diagnostic criteria, and recommendations.1

Narcolepsy Type 1 - Alternate names: hypocretin deficiency syndrome, narcolepsy-cataplexy, narcolepsy with cataplexy

Criteria A and B must be met:

  1. The patient has daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for ≥3 months.*
  2. The presence of one or both of the following:
    1. Cataplexy (as defined under Essential Features) and a mean sleep latency of ≤8 minutes and ≥2 sleep-onset REM periods (SOREMPs) on an MSLT performed according to standard techniques. A SOREMP (within 15 minutes of sleep onset) on the preceding nocturnal polysomnogram may replace one of the SOREMPs on the MSLT.
    2. CSF hypocretin-1 concentration, measured by immunoreactivity, is either ≤110 pg/mL or <1/3 of mean values obtained in normal subjects with the same standardized assay.
Notes:

* In young children, narcolepsy may sometimes present as excessively long night sleep or as resumption of previously discontinued daytime napping.

If narcolepsy type 1 is strongly suspected clinically but the MSLT criteria of B1 are not met, a possible strategy is to repeat the MSLT.

Reprinted with permission from the International Classification of Sleep Disorders. 3rd ed. (2014) American Academy of Sleep Medicine.

CSF = cerebrospinal fluid; MSLT = multiple sleep latency test; REM = rapid eye movement.


Narcolepsy Type 2 - Alternate name: narcolepsy without cataplexy

Criteria A-E must be met:

  1. The patient has daily periods of irrepressible need to sleep or daytime lapses into sleep occurring for ≥3 months.
  2. A mean sleep latency of ≤8 minutes and ≥2 sleep-onset REM periods (SOREMPs) are found on an MSLT performed according to standard techniques. 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. Either CSF hypocretin-1 concentration has not been measured or CSF hypocretin-1 concentration measured by immunoreactivity is either
    >110 pg/mL or >1/3 of mean values obtained in normal subjects with the same standardized assay.
  5. The hypersomnolence and/or MSLT findings are not better explained by other causes such as insufficient sleep, obstructive sleep apnea, delayed sleep phase disorder, or the effect of medication or substances or their withdrawal.
Notes:

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

If the CSF hypocretin-1 concentration is tested at a later stage and found to be either ≤110 pg/mL or <1/3 of mean values obtained in normal subjects with the same assay, then the disorder should be reclassified as narcolepsy type 1.

Reprinted with permission from the International Classification of Sleep Disorders. 3rd ed. (2014) American Academy of Sleep Medicine.

CSF = cerebrospinal fluid; MSLT = multiple sleep latency test; REM = rapid eye movement.


DSM-5 Diagnostic Criteria

The American Psychiatric Association (APA) Diagnostic and Statistical Manual of Mental Disorders – Fifth Edition (DSM-5), released in May 2013, contains diagnostic criteria for sleep-wake disorders, including narcolepsy, designed for use by mental health and medical clinicians who are not experts in sleep medicine.2

Additional information can be found at http://psychiatry.org/. This link is provided for the reader's information only; Jazz Pharmaceuticals does not endorse and is not responsible for the content of the website.

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