{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T01:10:20Z","timestamp":1777857020562,"version":"3.51.4"},"reference-count":23,"publisher":"SAGE Publications","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JND"],"published-print":{"date-parts":[[2022,1,4]]},"abstract":"<jats:p>Background: Spinal Muscular Atrophy (SMA) is the most common neurodegenerative disease in childhood. New therapeutic interventions have been developed to interrupt rapid motor deterioration. The current standard of clinical evaluation for severely weak infants is the Children\u2019s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND), originally developed for SMA type 1. This test however, remains subjective and requires extensive training to be performed reliably. Objective: Proof of principle of the motion tracking method for capturing complex movement patterns in ten children with SMA. Methods: We have developed a system for tracking full-body motion in infants (KineMAT) using a commercially available, low-cost RGB-depth sensor. Ten patients with SMA (2\u201346 months of age; CHOP INTEND score 10\u201350) were recorded for 2 minutes during unperturbed spontaneous whole-body activity. Five predefined motion parameters representing 56 degrees of freedom of upper, lower extremities and trunk joints were correlated with CHOP INTEND scores using Pearson product momentum correlation (r). Test-retest analysis in two patients used descriptive statistics. Results: 4\/5 preselected motion parameters highly correlated with CHOP INTEND: 1. Standard deviation of joint angles (r\u200a=\u200a0.959, test-retest range 1.3\u20131.9%), 2. Standard deviation of joint position (r\u200a=\u200a0.933, test-retest range 2.9%), 3. Absolute distance of hand\/foot travelled (r\u200a=\u200a0.937, test-retest range 6\u201310.5%), 4. Absolute distance of hand\/foot travelled against gravity (r\u200a=\u200a0.923; test-retest range 4.8\u20138.5%). Conclusions: Markerless whole-body motion capture using the KineMAT proved to objectively capture motor performance in infants and children with SMA across different severity and ages.<\/jats:p>","DOI":"10.3233\/jnd-200619","type":"journal-article","created":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T11:10:19Z","timestamp":1627038619000},"page":"121-128","source":"Crossref","is-referenced-by-count":6,"title":["Quantitative Motion Measurements Based on Markerless 3D Full-Body Tracking in Children with SMA Highly Correlate with Standardized Motor Assessments"],"prefix":"10.1177","volume":"9","author":[{"given":"Astrid","family":"Blaschek","sequence":"first","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nikolas","family":"Hesse","sequence":"additional","affiliation":[{"name":"Swiss Children\u2019s Rehab, University Children\u2019s Hospital Zurich, Affoltern am Albis, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Birgit","family":"Warken","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katharina","family":"Vill","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Therese","family":"Well","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Hodek","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florian","family":"Heinen","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"M\u00fcller-Felber","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas Sebastian","family":"Schroeder","sequence":"additional","affiliation":[{"name":"Ludwig Maximilian University of Munich (LMU), Hauner Children\u2019s Hospital, Paediatric Neurology and Developmental Medicine, Munich, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","reference":[{"issue":"1","key":"10.3233\/JND-200619_ref1","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"issue":"1","key":"10.3233\/JND-200619_ref2","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1038\/ejhg.2011.134","article-title":"Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72,400 specimens","volume":"20","author":"Sugarman","year":"2012","journal-title":"European Journal of Human Genetics: EJHG"},{"issue":"2","key":"10.3233\/JND-200619_ref3","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.nmd.2017.11.005","article-title":"Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care","volume":"28","author":"Mercuri","year":"2018","journal-title":"Neuromuscular Disorders: NMD"},{"issue":"3","key":"10.3233\/JND-200619_ref4","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.nmd.2017.11.004","article-title":"Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; 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