To split or to lump? Classifying the central disorders of hypersomnolence

Abstract The classification of the central disorders of hypersomnolence has undergone multiple iterations in an attempt to capture biologically meaningful disease entities in the absence of known pathophysiology. Accumulating data suggests that further refinements may be necessary. At the 7th International Symposium on Narcolepsy, a group of clinician-scientists evaluated data in support of keeping or changing classifications, and as a result suggest several changes. First, idiopathic hypersomnia with long sleep durations appears to be an identifiable and meaningful disease subtype. Second, idiopathic hypersomnia without long sleep time and narcolepsy without cataplexy share substantial phenotypic overlap and cannot reliably be distinguished with current testing, and so combining them into a single disease entity seems warranted at present. Moving forward, it is critical to phenotype patients across a wide variety of clinical and biological features, to aid in future refinements of disease classification.

[1]  F. Poli,et al.  Polysomnographic study of nocturnal sleep in idiopathic hypersomnia without long sleep time , 2013, Journal of sleep research.

[2]  I. Arnulf,et al.  Idiopathic hypersomnia with and without long sleep time: a controlled series of 75 patients. , 2009, Sleep.

[3]  E. Mignot,et al.  The MSLT is Repeatable in Narcolepsy Type 1 But Not Narcolepsy Type 2: A Retrospective Patient Study. , 2018, Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine.

[4]  E. Mignot,et al.  Nocturnal Sleep Dynamics Identify Narcolepsy Type 1. , 2015, Sleep.

[5]  P. Jennum,et al.  Sleep transitions in hypocretin-deficient narcolepsy. , 2013, Sleep.

[6]  Frits Koning,et al.  Narcolepsy — clinical spectrum, aetiopathophysiology, diagnosis and treatment , 2019, Nature Reviews Neurology.

[7]  R. O'Hara,et al.  Narcolepsy in African Americans. , 2015, Sleep.

[8]  T. Similowski,et al.  Residual sleepiness in obstructive sleep apnoea: phenotype and related symptoms , 2011, European Respiratory Journal.

[9]  J. H. Lee,et al.  Different fates of excessive daytime sleepiness: survival analysis for remission , 2016, Acta neurologica Scandinavica.

[10]  C. Gieger,et al.  Narcolepsy is strongly associated with the TCR alpha locus , 2009, Nature Genetics.

[11]  Sebastiaan Overeem,et al.  Clinical, polysomnographic and genome‐wide association analyses of narcolepsy with cataplexy: a European Narcolepsy Network study , 2013, Journal of sleep research.

[12]  I. Jaussent,et al.  Alternative diagnostic criteria for idiopathic hypersomnia: A 32‐hour protocol , 2018, Annals of neurology.

[13]  E. Mignot,et al.  Daytime continuous polysomnography predicts MSLT results in hypersomnias of central origin , 2013, Journal of sleep research.

[14]  E. Mignot,et al.  The autoimmune basis of narcolepsy , 2013, Current Opinion in Neurobiology.

[15]  M. Billiard,et al.  Narcolepsy with and without cataplexy, idiopathic hypersomnia with and without long sleep time: a cluster analysis. , 2015, Sleep medicine.

[16]  Y. Harrison,et al.  “High sleepability without sleepiness”. The ability to fall asleep rapidly without other signs of sleepiness , 1996, Neurophysiologie Clinique/Clinical Neurophysiology.

[17]  J. Golmard,et al.  Lucid dreaming in narcolepsy. , 2015, Sleep.

[18]  R. Howard,et al.  REM sleep behaviour disorder (RBD) and its associations in young patients. , 2009, Sleep medicine.

[19]  B. Becher,et al.  T cells in patients with narcolepsy target self-antigens of hypocretin neurons , 2018, Nature.

[20]  C. Guilleminault,et al.  HLA DQB1*0602 is associated with cataplexy in 509 narcoleptic patients. , 1997, Sleep.

[21]  I. Arnulf,et al.  Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness , 2010, Journal of sleep research.

[22]  E. Mignot,et al.  Challenges in diagnosing narcolepsy without cataplexy: a consensus statement. , 2014, Sleep.

[23]  K. Maski,et al.  Nocturnal REM Sleep Without Atonia Is a Diagnostic Biomarker of Pediatric Narcolepsy. , 2018, Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine.

[24]  G. Plazzi,et al.  Decreased sleep stage transition pattern complexity in narcolepsy type 1 , 2016, Clinical Neurophysiology.

[25]  J. Backhaus,et al.  A 19‐h spontaneous sleep period in idiopathic central nervous system hypersomnia , 1998, Journal of sleep research.

[26]  E. Matsushima,et al.  Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1( *)1501/DQB1( *)0602 finding. , 2009, Sleep medicine.

[27]  I. Jaussent,et al.  Test–Retest Reliability of the Multiple Sleep Latency Test in Central Disorders of Hypersomnolence , 2017, Sleep.

[28]  L. Trotti,et al.  Waking up is the hardest thing I do all day: Sleep inertia and sleep drunkenness. , 2017, Sleep medicine reviews.

[29]  T. Young,et al.  Nocturnal rapid eye movement sleep latency for identifying patients with narcolepsy/hypocretin deficiency. , 2013, JAMA neurology.

[30]  M. Thorpy,et al.  International classification of sleep disorders , 2017 .

[31]  M. Vidailhet,et al.  Hallucinations in narcolepsy with and without cataplexy: contrasts with Parkinson's disease. , 2011, Sleep medicine.

[32]  Y. Inoue,et al.  Differences in electroencephalographic findings among categories of narcolepsy-spectrum disorders. , 2015, Sleep medicine.

[33]  D. Rye,et al.  Test-retest reliability of the multiple sleep latency test in narcolepsy without cataplexy and idiopathic hypersomnia. , 2013, Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine.

[34]  L. Sharples,et al.  Idiopathic hypersomnia: a study of 77 cases. , 2007, Sleep.

[35]  A. Rechtschaffen,et al.  Hypersomnia with "sleep drunkenness". , 1972, Archives of general psychiatry.

[36]  E. Mignot,et al.  Primary progressive narcolepsy type 1: The other side of the coin , 2014, Neurology.

[37]  M. Aldrich,et al.  Idiopathic hypersomnia : A series of 42 patients , 1997 .

[38]  E. Mignot,et al.  Predictors of hypocretin (orexin) deficiency in narcolepsy without cataplexy. , 2012, Sleep.

[39]  I. Arnulf,et al.  Narcolepsy with long sleep time: a specific entity? , 2009, Sleep.

[40]  I. Jaussent,et al.  Temporal Changes in the Cerebrospinal Fluid Level of Hypocretin-1 and Histamine in Narcolepsy , 2017, Sleep.

[41]  B. Roth Narcolepsy and hypersomnia: review and classification of 642 personally observed cases. , 1976, Schweizer Archiv fur Neurologie, Neurochirurgie und Psychiatrie = Archives suisses de neurologie, neurochirurgie et de psychiatrie.

[42]  Michael L. Prairie,et al.  Optimizing Actigraphic Estimation of Sleep Duration in Suspected Idiopathic Hypersomnia. , 2019, Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine.

[43]  A. Harvey,et al.  Development and Validation of the Sleep Inertia Questionnaire (SIQ) and Assessment of Sleep Inertia in Analogue and Clinical Depression , 2015, Cognitive Therapy and Research.