1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-5-90-a 10.15844/pedneurbriefs-5-12-2 Congenital Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Prader-Willi Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 12 1991 01 07 2016 5 12 90 90 Copyright: © 1991 The Author(s) 1991 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Sleep and breathing in patients with the Prader-Willi syndrome

The clinical and polysomnographic features of 5 patients with Prader-Willi syndrome were studied at the Mayo Clinic, Jacksonville, FL.

 Prader-Willi Syndrome Hypersomnolence Anesthesia

The clinical and polysomnographic features of 5 patients with Prader-Willi syndrome were studied at the Mayo Clinic, Jacksonville, FL. All patients, including 2 adults, 1 adolescent, and 2 children were obese; they had hypersomnolence and daytime hypoxemia, and they snored. The apnea plus hyponea index was less than 10 episodes per hour of sleep. Polysomnography failed to demonstrate clinically severe obstructive apnea or hypopnea. Sleep architecture and rapid eye movement latency were normal in all patients. Nonapneic reductions in oxyhemoglobin saturation were detected in only 1 adult and in 1 child. Despite the presence of morbid obesity and a history of snoring these patients with Prader-Willi syndrome had only mild sleep-disordered breathing. [1]

COMMENT. Prader-Willi syndrome is characterized by infantile hypotonia, early childhood obesity, mental deficiency, short stature, small hands and feet and hypogonadism. Many patients also have hypersomnolence, daytime hypoventilation, and some die prematurely of cardiorespiratory failure. The epidemiology of Prader-Willi syndrome was studied in a rural county in Sweden and reported from the Department of Pediatrics and Child Psychiatry, Child Neuropsychiatry Centre, Goteborg, Sweden [2]. For the 7-25 year old age group the prevalence of Prader-Willi syndrome was 13 per 100,000 for definite cases, and 20 per 100,000 if borderline cases were included.

Anesthesia and the Prader-Willi syndrome was the subject of a report from the John Radcliffe Hospital, Oxford, England [3]. A 2 year old boy presented for correction of ventral curvature of micropenis and an 18 year old received anesthetic for removal of excess tissue from one thigh. Halothane in nitrous oxide and oxygen were used for induction and isoflurane to maintain anesthesia. Attention to glycaemic control and body temperature is important since glucose intolerance and both hypothermia and hyperthermia have been reported in response to operative stress.

 Kaplan J Fredrickson PA Richardson JW Sleep and breathing in patients with the Prader-Willi syndrome Mayo Clin Proc 1991 Nov 66 11 1124 1126 10.1016/S0025-6196(12)65792-9 1943244 Akefeldt A Gillberg C Larsson C Prader-Willi syndrome in a Swedish rural county: epidemiological aspects Dev Med Child Neurol 1991 Aug 33 8 715 721 10.1111/j.1469-8749.1991.tb14950.x 1916026 Mackenzie JW Anaesthesia and the Prader-Willi syndrome J R Soc Med 1991 Apr 84 4 239 2027156