TABLE 3-3International Classification of Sleep Disorders: Definitions, Prevalence, and Pathophysiology of Narcolepsy and Hypersomnias

Condition Diagnostic Criteria North American Prevalence Pathophysiology and Etiology
Narcolepsy with cataplexyPresence of definite cataplexy; usually abnormal MSLT results0.02–0.18%Hypocretin deficiency; 90% with low CSF HCRT-1 and positive for HLA-DQB1*0602
Narcolepsy without cataplexyMSLT: mean sleep latency less than or equal to 8 minutes, 2 or greater SOREMPs; no or doubtful cataplexy0.02%; many undiagnosedUnknown, probably heterogeneous; 7– 25% with low CSF HCRT-1, 40% HLA-DQB1*0602 positive
Secondary narcolepsy or hypersomniaAs above, but due to other known medical conditions (e.g., neurological)UnknownWith or without hypocretin deficiency
Idiopathic hypersomnia with prolonged sleepMSLT: short mean sleep latency, greater than two SOREMPs; long (10 hours or greater) unrefreshing nocturnal sleepRare, maybe 0.01–0.02%Unknown, probable heterogeneous etiology
Idiopathic hypersomnia with normal sleep lengthMSLT: short mean sleep latency, less than two SOREMPs; normal nightly sleep amounts (less than 10 hours)Probably common, unknown prevalenceUnknown, probable heterogeneous etiology
Periodic hypersomnia (includes Kleine-Levin syndrome)Recurrent (more than 1 time per year) sleepiness (lasting 2 to 28 days), normal function between occurrencesRare, probably less than one per one million peopleUnknown, probable heterogeneous etiology

NOTE: CSF, lumbar sac cerebrospinal fluid; HCRT, hypocretin; HLA, human leukocyte antigen; MSLT, Multiple Sleep Latency Test; SOREMP, sleep-onset REM period.

SOURCE: AASM (2005).

From: 3, Extent and Health Consequences of Chronic Sleep Loss and Sleep Disorders

Cover of Sleep Disorders and Sleep Deprivation
Sleep Disorders and Sleep Deprivation: An Unmet Public Health Problem.
Institute of Medicine (US) Committee on Sleep Medicine and Research; Colten HR, Altevogt BM, editors.
Washington (DC): National Academies Press (US); 2006.
Copyright © 2006, National Academy of Sciences.

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