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

Author:

Fronczek Rolf12ORCID,Arnulf Isabelle3ORCID,Baumann Christian R4,Maski Kiran5,Pizza Fabio67,Trotti Lynn Marie8ORCID

Affiliation:

1. Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands

2. Sleep-Wakecentre SEIN, Heemstede, The Netherlands

3. Sorbonne University, National Reference Center for Rare Hypersomnia, Pitie-Salpetriere Hospital, Paris, France

4. Department of Neurology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

5. Department of Neurology, Harvard Medical School, Boston Children’s Hospital, Boston, MA

6. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy

7. IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

8. Department of Neurology, Emory University School of Medicine, Atlanta, GA

Abstract

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.

Funder

National Institutes of Health

Publisher

Oxford University Press (OUP)

Subject

Physiology (medical),Clinical Neurology

Reference46 articles.

1. Narcolepsy and hypersomnia: review and classification of 642 personally observed cases;Roth;Schweiz Arch Neurol Neurochir Psychiatr.,1976

2. Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness;Vernet;J Sleep Res.,2010

3. HLA DQB1*0602 is associated with cataplexy in 509 narcoleptic patients;Mignot;Sleep.,1997

4. Narcolepsy is strongly associated with the T-cell receptor alpha locus;Hallmayer;Nat Genet.,2009

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