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Diagnostic criteria for narcolepsy type 1 (ICSD-3-TR)

Diagnostic criteria for narcolepsy type 1 (ICSD-3-TR)
Criteria A through C must be met:
A The patient has daily periods of irrepressible need to sleep or daytime lapses into drowsiness or sleep.
B The presence of one or both of the following:
  1. Cataplexy* 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 ≤110 pg/mL (using a Stanford reference sample) or <1/3 of mean values obtained in normal subjects with the same standardized assayΔ
C 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.

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

* Typical cataplexy is most strongly associated with narcolepsy type 1 (NT1). Although some patients with NT1/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 NT1. Clinical judgment is required. Further guidance regarding distinguishing typical from atypical cataplexy is included in the Essential Features section of the International Classification of Sleep Disorders, third edition, text revision (ICSD-3-TR).

¶ Hypocretin-1 is also known as orexin-A.

Δ If hypocretin deficiency is verified, the diagnosis of NT1 should be made regardless of other comorbidities that could potentially be related to clinical symptoms, given the definitive nature of this finding.
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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