Def: recurrent episodes of hypersomnia with cognitive and/or behavioral changesVery rare, prevalence 1-5 per millionMale predominanceResolves in median 13-14 yrs, may persist for decades EpisodesHallmark: discrete attacks of hypersomnolence with abrupt onset over a few hrsLast days to weeksPt appears sleepy, confused, apathetic or dream-likeSleep up to 22 hrs/24 hrsVery hard to wake, near end… Continue reading →

Rare chronic CNS d/o with clinical overlap with narcolepsy~10x less common than narcolepsyPoorly defined, may be a heterogeneous group of d/oOnset at any age, most often adolescence or early adulthood Clinical featuresMarked EDS is the core featureLong sleep duration, typically ≥10 hrs for main sleep periodLong unrefreshing naps, usually ≥1 hrSleep inertia (sleep drunkenness): marked… Continue reading →

Def: chronic neurological dz sharing PSG and many clinical features with NT1Cataplexy absent, CSF hypocretin-1 not proven lowClinical presentation similar to NT1 but without cataplexyAge of onset and sex distribution mirror NT1 PathophysiologyUnclear~45% HLA-DQB1*06:02 positive Dx criteria (ICSD-3, all required) Differential dx TreatmentSimilar to NT1, minus cataplexy txUS: sodium oxybate, low-sodium oxybate, pitolisant and solriamfetol… Continue reading →

Def: chronic neurological dz caused by extensive loss of hypothalamic orexin (hypocretin) A and B neurons Cardinal features Other featuresDisturbed nighttime sleep with increased N1Brief naps of minutes are usually refreshingComorbid sleep d/o: RBD, OSA, PLMSDepression and anxiety, cause unclear: QoL impact or neurochemical changes Epidemiology: Prevalence 1 per 2000-4000 (0.025-0.05%) worldwideBimodal onset: peak ~15… Continue reading →