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

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 n...

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Autores principales: Weselake, Sara V, Foulds, Jessica L, Couch, Robert, Witmans, Manisha B, Rubin, Daniela, Haqq, Andrea M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4000141/
https://www.ncbi.nlm.nih.gov/pubmed/24742112
http://dx.doi.org/10.1186/1752-1947-8-127
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author Weselake, Sara V
Foulds, Jessica L
Couch, Robert
Witmans, Manisha B
Rubin, Daniela
Haqq, Andrea M
author_facet Weselake, Sara V
Foulds, Jessica L
Couch, Robert
Witmans, Manisha B
Rubin, Daniela
Haqq, Andrea M
author_sort Weselake, Sara V
collection PubMed
description 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.
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spelling pubmed-40001412014-04-26 Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report Weselake, Sara V Foulds, Jessica L Couch, Robert Witmans, Manisha B Rubin, Daniela Haqq, Andrea M J Med Case Rep Case Report 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. BioMed Central 2014-04-17 /pmc/articles/PMC4000141/ /pubmed/24742112 http://dx.doi.org/10.1186/1752-1947-8-127 Text en Copyright © 2014 Weselake et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.
spellingShingle Case Report
Weselake, Sara V
Foulds, Jessica L
Couch, Robert
Witmans, Manisha B
Rubin, Daniela
Haqq, Andrea M
Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title_full Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title_fullStr Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title_full_unstemmed Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title_short Prader-Willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
title_sort prader-willi syndrome, excessive daytime sleepiness, and narcoleptic symptoms: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4000141/
https://www.ncbi.nlm.nih.gov/pubmed/24742112
http://dx.doi.org/10.1186/1752-1947-8-127
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