Biomaterial Database

Prader Willi Syndrome and excessive daytime sleepiness. 2008

Danny Camfferman, and R Doug McEvoy, and Fergal O'Donoghue, and Kurt Lushington

Adelaide Institute for Sleep Health, Repatriation General Hospital, Daw Park, South Australia 5042, Australia.

Prader Willi Syndrome (PWS) is a rare genetic disorder characterized by a range of physical, psychological and physiological abnormalities. PWS patients may also demonstrate a range of abnormalities of sleep architecture and of breathing during sleep, and excessive daytime sleepiness (EDS). In the general population EDS is associated with Obstructive Sleep Apnea Syndrome (OSAS). In PWS, by contrast, OSAS is unlikely to fully explain EDS and other factors, including hypothalamic dysfunction are likely to contribute. The present review examines OSAS and hypothalamic dysfunction and other contributing factors to EDS in PWS.

UI	MeSH Term	Description	Entries
D006970	Disorders of Excessive Somnolence	Disorders characterized by hypersomnolence during normal waking hours that may impair cognitive functioning. Subtypes include primary hypersomnia disorders (e.g., IDIOPATHIC HYPERSOMNOLENCE; NARCOLEPSY; and KLEINE-LEVIN SYNDROME) and secondary hypersomnia disorders where excessive somnolence can be attributed to a known cause (e.g., drug affect, MENTAL DISORDERS, and SLEEP APNEA SYNDROME). (From J Neurol Sci 1998 Jan 8;153(2):192-202; Thorpy, Principles and Practice of Sleep Medicine, 2nd ed, p320)	Daytime Sleepiness,Daytime Somnolence,Excessive Daytime Sleepiness,Hypersomnia,Hypersomnolence,Primary Hypersomnia Disorders,Secondary Hypersomnia Disorders,DOES (Disorders of Excessive Somnolence),Excessive Somnolence Disorders,Hypersomnia, Recurrent,Hypersomnolence Disorders,Hypersomnolence Disorders, Primary,Hypersomnolence Disorders, Secondary,Primary Hypersomnolence Disorders,Secondary Hypersomnolence Disorders,DOESs (Disorders of Excessive Somnolence),Daytime Sleepiness, Excessive,Daytime Sleepinesses,Daytime Somnolences,Excessive Daytime Sleepinesses,Excessive Somnolence Disorder,Hypersomnia Disorder, Primary,Hypersomnia Disorder, Secondary,Hypersomnias,Hypersomnolence Disorder,Hypersomnolence Disorder, Primary,Hypersomnolence Disorder, Secondary,Primary Hypersomnia Disorder,Primary Hypersomnolence Disorder,Recurrent Hypersomnia,Recurrent Hypersomnias,Secondary Hypersomnia Disorder,Secondary Hypersomnolence Disorder,Sleepiness, Daytime,Sleepiness, Excessive Daytime,Somnolence Disorder, Excessive,Somnolence, Daytime
D007031	Hypothalamus	Ventral part of the DIENCEPHALON extending from the region of the OPTIC CHIASM to the caudal border of the MAMMILLARY BODIES and forming the inferior and lateral walls of the THIRD VENTRICLE.	Lamina Terminalis,Preoptico-Hypothalamic Area,Area, Preoptico-Hypothalamic,Areas, Preoptico-Hypothalamic,Preoptico Hypothalamic Area,Preoptico-Hypothalamic Areas
D011218	Prader-Willi Syndrome	An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229)	Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D012307	Risk Factors	An aspect of personal behavior or lifestyle, environmental exposure, inborn or inherited characteristic, which, based on epidemiological evidence, is known to be associated with a health-related condition considered important to prevent.	Health Correlates,Risk Factor Scores,Risk Scores,Social Risk Factors,Population at Risk,Populations at Risk,Correlates, Health,Factor, Risk,Factor, Social Risk,Factors, Social Risk,Risk Factor,Risk Factor Score,Risk Factor, Social,Risk Factors, Social,Risk Score,Score, Risk,Score, Risk Factor,Social Risk Factor
D015992	Body Mass Index	An indicator of body density as determined by the relationship of BODY WEIGHT to BODY HEIGHT. BMI	Quetelet Index,Quetelet's Index,Index, Body Mass,Index, Quetelet,Quetelets Index
D017286	Polysomnography	Simultaneous and continuous monitoring of several parameters during sleep to study normal and abnormal sleep. The study includes monitoring of brain waves, to assess sleep stages, and other physiological variables such as breathing, eye movements, and blood oxygen levels which exhibit a disrupted pattern with sleep disturbances.	Monitoring, Sleep,Somnography,Polysomnographies,Sleep Monitoring,Somnographies