Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report

Simple item page
dc.contributor.authorWeselake, Sara V
dc.contributor.authorFoulds, Jessica L
dc.contributor.authorCouch, Robert
dc.contributor.authorWitmans, Manisha B
dc.contributor.authorRubin, Daniela
dc.contributor.authorHaqq, Andrea M
dc.date.accessioned2014-04-25T19:02:33Z
dc.date.available2014-04-25T19:02:33Z
dc.date.issued2014-04-17
dc.date.updated2014-04-25T19:02:33Z
dc.description.abstractAbstract Introduction Sleep abnormalities, including narcolepsy and cataplexy, are a common feature of Prader-Willi syndrome. Long-term treatment with the central nervous system stimulant modafinil has not been reported. In this case report we present a longitudinal perspective of sleep abnormalities in a nine-year-old Caucasian girl with Prader-Willi syndrome from age two to age nine, and detail the response to treatment with the central nervous system stimulant modafinil. Case presentation Our patient presented at two years of age with hypersomnia and narcoleptic episodes with cataplectic features. Initial polysomnograph testing revealed adequate sleep efficiency, but increased sleep fragmentation especially during rapid eye movement sleep. The narcoleptic episodes continued and a repeat polysomnograph at age five years confirmed features consistent with narcolepsy. Further sleep studies at six years, including a multiple sleep latency test, demonstrated signs of excessive daytime sleepiness. Treatment with modafinil was initiated at age seven years six months due to persistent hypersomnia and narcoleptic symptoms. Two polysomnograph studies were performed following treatment with modafinil, at age eight years six months and nine years three months. These studies showed excellent sleep efficiency and improvement of rapid eye movement sleep parameters, supporting the beneficial effects of long-term modafinil therapy. Conclusions Long-term modafinil therapy may ameliorate the sleep disturbances of Prader-Willi syndrome and should be the focus of future clinical trials.
dc.description.versionPeer Reviewed
dc.identifier.citationJournal of Medical Case Reports. 2014 Apr 17;8(1):127
dc.identifier.doihttp://dx.doi.org/10.1186/1752-1947-8-127
dc.identifier.urihttp://hdl.handle.net/1993/23559
dc.language.rfc3066en
dc.rightsopen accessen_US
dc.rights.holderSara V Weselake et al.; licensee BioMed Central Ltd.
dc.titlePrader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
dc.typeJournal Article
Files
Original bundle
Now showing 1 - 2 of 2
Thumbnail Image
Name:
1752-1947-8-127.xml
Size:
45.41 KB
Format:
Extensible Markup Language
Description:
Download
Thumbnail Image
Name:
1752-1947-8-127.pdf
Size:
363.25 KB
Format:
Adobe Portable Document Format
Description:
Download
License bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
license.txt
Size:
2.17 KB
Format:
Item-specific license agreed to upon submission
Description:
Download
Collections
University of Manitoba Scholarship
Rady Faculty of Health Sciences Scholarly Works