Polysomnography findings in pediatric spinal muscular atrophy types 1-3. Chacko, A, Sly, Peter and Gauld, L 2020, Polysomnography findings in pediatric spinal muscular atrophy types 1-3., Sleep Med, vol. 68, pp. 124-130, doi: 10.1016/j.sleep.2019.12.004. Title Polysomnography findings in pediatric spinal muscular atrophy types 1-3. Author(s) Chacko, A Sly, Peter Gauld, L Journal name Sleep Med Volume number 68 Start page 124 End page 130 Total pages 7 Publisher p Place of publication Netherlands Publication date 2020-04 ISSN 1878-5506 Keyword(s) Science & Technology Life Sciences & Biomedicine Clinical Neurology Neurosciences & Neurology Pediatric spinal muscular atrophy Polysomnography Spinal muscular atrophy type one Spinal muscular atrophy type two Spinal muscular atrophy type three NONINVASIVE VENTILATION SLEEP ARCHITECTURE CHILDREN INFANTS DISEASE VALUES MANAGEMENT DISORDERS PULMONARY MECHANICS Summary BACKGROUND: Sleep disordered breathing (SDB) causes sleep disturbance and daytime symptoms in children with neuromuscular disorders. Although polysomnography (PSG) findings are well described in many neuromuscular disorders, there are limited reports from children with spinal muscular atrophy (SMA). The aim of this study was to determine the sleep architecture and breathing characteristics and non-invasive ventilation (NIV) use in our pediatric SMA cohort. METHODS: We conducted a cross-sectional cohort study of all children with SMA in Queensland, Australia. Children were Nusinersen naïve and had a full diagnostic PSG in 2018. The PSG was scored and reported by a single pediatric sleep physician in accordance with American Academy of Sleep Medicine Criteria (2012). RESULTS: In sum, 31 children (18 males), Six with Type 1, 16 with Type 2 and nine with Type 3, aged 0.25-18.8 years old were studied. SDB was seen in each SMA type and was more pronounced during rapid eye movement (REM) sleep. Type 1: all patients exhibited SDB, three (50%) with central sleep apnea (CSA) and three (50%) with mixed disease. Type 2: five (31%) had CSA, one (6%) mixed disease, seven (44%) had early SDB and three (19%) had normal sleep breathing. Type 3: four (44%) children had CSA and five had early SDB. No child exhibited obstructive sleep apnea (OSA) alone.Starting NIV significantly reduced mean total PSG Apnea-Hypopnea Index (AHI) scores from a grouped mean of 15.4 events per hour (SD ± 14.6; 95% CI 6.1-24.7) to 4.0 events per hour (SD ± 4.2, 95% CI 1.2-6.5, p = 0.01). CONCLUSION: SDB is common in children with SMA and was present in all types. CSA was the most common disorder; with mixed SDB also present in type 1 and 2 SMA. Language eng DOI 10.1016/j.sleep.2019.12.004 Indigenous content off Field of Research 1103 Clinical Sciences 1701 Psychology Persistent URL http://hdl.handle.net/10536/DRO/DU:30164627 Document type: Journal Article Collections: Faculty of Health School of Medicine Related Links Link Description Connect to published version Go to link with your DU access privileges   Author URL Go to link with your DU access privileges   Connect to Elements publication management system Go to link with your DU access privileges     Unless expressly stated otherwise, the copyright for items in DRO is owned by the author, with all rights reserved. Versions Version Filter Type Fri, 18 Mar 2022, 07:35:40 EST Fri, 18 Mar 2022, 15:59:07 EST Wed, 06 Apr 2022, 08:59:12 EST Thu, 21 Apr 2022, 15:54:57 EST Thu, 21 Apr 2022, 16:31:55 EST Filtered Full Citation counts:   Cited 5 times in TR Web of Science Cited 6 times in Scopus Search Google Scholar Access Statistics: 6 Abstract Views  -  Detailed Statistics Created: Fri, 18 Mar 2022, 07:35:40 EST