ICD-11 code : 7A20.0;
Preferred Label : Narcolepsy, Type 1;
ICD-11 definition : Type 1 narcolepsy is a disorder of excessive sleepiness due to a deficiency of hypothalamic
hypocretin (orexin) signaling. In addition to daily periods of irrepressible need
to sleep or daytime lapses into sleep, type 1 narcolepsy is characterised by symptoms
of REM sleep dissociation, most importantly cataplexy. Cataplexy is a sudden and uncontrollable
loss of muscle tone arising during wakefulness that is typically triggered by a strong
emotion, such as excitement or laughter. Although cataplexy is a pathognomonic symptom
of type 1 narcolepsy, it may not manifest until years following onset of the sleepiness.
In such cases, a diagnosis of narcolepsy, type 1 may be made based on cerebrospinal
fluid (CSF)-hypocretin levels 110 picograms per milliliter. Episodes of sleep paralysis
and hypnagogic or hypnopompic hallucinations may also be present. The disorder is
not attributable to a disease of the nervous system or other medical condition. Note:
A definitive diagnosis requires daily periods of irrepressible need to sleep or daytime
lapses into sleep plus either: a) cataplexy and multiple sleep latency test/polysomnography
(MSLT/PSG) findings characteristic of narcolepsy; or b) demonstrated CSF hypocretin
deficiency.;
ICD-11 synonym : Narcolepsy with cataplexy and/or hypocretin deficiency; cataplexy; Gélineau's syndrome;
Origin ID : 1851015159;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
- Validated automatic mappings to NTBT
Type 1 narcolepsy is a disorder of excessive sleepiness due to a deficiency of hypothalamic
hypocretin (orexin) signaling. In addition to daily periods of irrepressible need
to sleep or daytime lapses into sleep, type 1 narcolepsy is characterised by symptoms
of REM sleep dissociation, most importantly cataplexy. Cataplexy is a sudden and uncontrollable
loss of muscle tone arising during wakefulness that is typically triggered by a strong
emotion, such as excitement or laughter. Although cataplexy is a pathognomonic symptom
of type 1 narcolepsy, it may not manifest until years following onset of the sleepiness.
In such cases, a diagnosis of narcolepsy, type 1 may be made based on cerebrospinal
fluid (CSF)-hypocretin levels 110 picograms per milliliter. Episodes of sleep paralysis
and hypnagogic or hypnopompic hallucinations may also be present. The disorder is
not attributable to a disease of the nervous system or other medical condition. Note:
A definitive diagnosis requires daily periods of irrepressible need to sleep or daytime
lapses into sleep plus either: a) cataplexy and multiple sleep latency test/polysomnography
(MSLT/PSG) findings characteristic of narcolepsy; or b) demonstrated CSF hypocretin
deficiency.
