Sleep disturbance in mucopolysaccharidosis type III (Sanfilippo syndrome): a survey of managing clinicians.

Title

Sleep disturbance in mucopolysaccharidosis type III (Sanfilippo syndrome): a survey of managing clinicians.

Creator

Fraser J; Wraith JE; Delatycki MB

Publisher

Clinical Genetics

Date

2002

Subject

Data Collection; Physicians; Humans; Behavior Therapy; Mucopolysaccharidosis III/complications; Sleep Disorders/complications; Sleep Disorders/drug therapy; Sleep Disorders/therapy; NET Files; sleep disturbance/disorders; MPS III; trajectory; characteristics

Description

Sanfilippo syndrome (mucopolysaccharidosis type III) is the commonest mucoploysaccharidosis. It causes neurodegeneration with often profound sleep and behavioral disturbance. Management of the sleep disturbance is difficult and inconsistent. In this study, we surveyed clinicians with particular expertise in the management of individuals with mucopolysaccharidoses. We found that sleep problems are almost universal in this patient population and that no one treatment is consistently viewed as beneficial. Among the clinicians surveyed, melatonin is reported as the medication most likely to be of benefit. Benzodiazepines, chloral hydrate, antihistamines and antipsychotic agents are overall reported as less efficacious. The major side-effect of the medications as a group was reported to be daytime somnolence. Based on this study, recommendations are given regarding the approach to sleep disturbance in Sanfilippo syndrome.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Fraser J; Wraith JE; Delatycki MB, “Sleep disturbance in mucopolysaccharidosis type III (Sanfilippo syndrome): a survey of managing clinicians.,” Pediatric Palliative Care Library, accessed October 17, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16656.

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