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Diagnostic criteria for narcolepsy type 2 (ICSD-3-TR)

Diagnostic criteria for narcolepsy type 2 (ICSD-3-TR)
Criteria A through E must be met:
A The patient has daily periods of irrepressible need to sleep or daytime lapses into drowsiness or sleep occurring for at least three months.
B A mean sleep latency of ≤8 minutes and two or more SOREMPs are found on an MSLT performed in accordance with current recommended protocols. A SOREMP (within 15 minutes of sleep onset) on the preceding nocturnal PSG may replace one of the SOREMPs on the MSLT.
C Cataplexy is absent.Δ
D If CSF hypocretin-1 concentration is measured by radioimmunoassay, it 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.
E 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/substances use or withdrawal.

CSF: cerebrospinal fluid; MSLT: multiple sleep latency test; PSG: polysomnography; SOREMPs: sleep-onset rapid eye movement periods.

* The diagnostic value of a SOREMP on nocturnal PSG 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.

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

◊ If the CSF hypocretin-1 (also known as orexin-A) 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, the disorder should be reclassified as narcolepsy type 1.
Reproduced with permission from: International Classification of Sleep Disorders, 3rd ed, text revision (ICSD-3-TR), American Academy of Sleep Medicine 2023. Copyright © 2023 American Academy of Sleep Medicine.
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