دورية أكاديمية
Upper Limb Evaluation and One-Year Follow Up of Non-Ambulant Patients with Spinal Muscular Atrophy: An Observational Multicenter Trial
| العنوان: | Upper Limb Evaluation and One-Year Follow Up of Non-Ambulant Patients with Spinal Muscular Atrophy: An Observational Multicenter Trial |
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| المؤلفون: | Seferian, Andreea Mihaela, Moraux, Amelie, Canal, Aurélie, Decostre, Valérie, Diebate, Oumar, Le Moing, Anne-Gaëlle, Gidaro, Teresa, Deconinck, Nicolas, Van Parys, Frauke, Vereecke, Wendy, Wittevrongel, Sylvia, Annoussamy, Melanie, Mayer, Michele, Maincent, Kim, Cuisset, Jean-Marie, Tiffreau, Vincent, Denis, Severine, Jousten, Virginie, Quijano-Roy, Susana, Voit, Thomas, Hogrel, Jean-Yves, Servais, Laurent |
| المساهمون: | Univ. Littoral Côte d’Opale, Univ. Artois, Université de Lille, Centre de Recherche en Informatique, Signal et Automatique de Lille (CRIStAL) - UMR 9189, Centre de recherche en myologie, CHU Amiens-Picardie, Universiteit Gent Ghent, CHU Trousseau APHP, Centre Hospitalier Régional Universitaire Lille CHRU Lille, Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - EA 7369, Centre Hospitalier Universitaire de Liège CHU-Liège, Université de Versailles Saint-Quentin-en-Yvelines UVSQ |
| سنة النشر: | 2020 |
| المجموعة: | LillOA (Lille Open Archive - Université de Lille) |
| مصطلحات موضوعية: | Mesh:Noninvasive Ventilation, Mesh:Muscular Atrophy, Mesh:Spinal/physiopathology, Mesh:Young Adult, Mesh:Upper Extremity/physiopathology, Mesh:Adolescent, Mesh:Adult, Mesh:Child, Mesh:Female, Mesh:Follow-Up Studies, Mesh:Hand Strength/physiology, Mesh:Humans, Mesh:Spinal Muscular Atrophies of Childhood/physiopathology, Mesh:Pinch Strength/physiology, Mesh:Male |
| الوصف: | Assessment of the upper limb strength in non-ambulant neuromuscular patients remains challenging. Although potential outcome measures have been reported, longitudinal data demonstrating sensitivity to clinical evolution in spinal muscular atrophy patients are critically lacking. Our study recruited 23 non-ambulant patients, 16 patients (males/females = 6/10; median age 15.4 years with a range from 10.7 to 31.1 years) with spinal muscular atrophy type II and 7 patients (males/females = 2/5; median age 19.9 years with a range from 8.3 to 29.9 years) with type III. The Brooke functional score was on median 3 with a range from 2 to 6. The average total vital capacity was 46%, and seven patients required non-invasive ventilation at night. Patients were assessed at baseline, 6 months, and 1 year using the Motor Function Measure and innovative devices MyoGrip, MyoPinch, and MoviPlate, which assess handgrip strength, key pinch strength, and hand/finger extension-flexion function, respectively. The study demonstrated the feasibility and reliability of these measures for all patients, and sensitivity to negative changes after the age of 14 years. The younger patients showed an increase of the distal force in the follow-up period. The distal force measurements and function were correlated to different functional scales. These data represent an important step in the process of validating these devices as potential outcome measures for future clinical trials. ; 10 |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/octet-stream |
| اللغة: | English |
| العلاقة: | PLoS One; http://hdl.handle.net/20.500.12210/12277.3Test |
| الإتاحة: | https://doi.org/20.500.12210/12277.3Test https://hdl.handle.net/20.500.12210/12277.3Test |
| حقوق: | Attribution 3.0 United States ; info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.C571CDC2 |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |