title: Upper limb strength and function changes during a one-year follow-up in non-ambulant patients with Duchenne Muscular Dystrophy: an observational multicenter trial.
abstract: BACKGROUND
  Upper limb evaluation of patients with Duchenne Muscular Dystrophy is crucially important to evaluations of efficacy of new treatments in non-ambulant patients. In patients who have lost ambulation, there are few validated and informative outcome measures. In addition, longitudinal data demonstrating sensitivity to clinical evolution of outcome measures over short-term periods are lacking.
  
  
  METHODS
  We report here the results of a one-year multicenter study using specifically designed tools to assess grip, pinch strength, and hand function in wheelchair-bound patients. Our study assessed 53 non-ambulant patients with Duchenne muscular dystrophy aged 17.1 ± 4.8 years (range: 9 - 28.1 years). The average Brooke functional score of these patients was 4.6 ± 1.1. The average forced vital capacity was 44.5% predicted and 19 patients used non-invasive ventilation. Patients were assessed at baseline, 6 months, and one year using the Motor Function Measure and innovative devices (namely the MyoSet composed of MyoGrip, MyoPinch, and MoviPlate).
  
  
  RESULTS
  Our study confirmed preliminary data previously reported regarding feasibility of use and of reliability of the MyoSet and the correlation at baseline between distal strength and clinical outcomes such as FVC, Brooke score, age, and duration since loss of ambulation. A significant correlation was observed between the distal upper limb strength and clinical variables. The sensitive dynamometers (MyoGrip and MyoPinch) and MoviPlate captured a 12-month change in non-ambulant Duchenne muscular dystrophy patients of all ages.
  
  
  BACKGROUND
  ClinicalTrials.gov NCT00993161 NCT00993161.
authors: Institute of Myology, Paris, France.
authors: Andreea Mihaela
authors: AM
authors: Seferian
authors: Institute of Myology, Paris, France.
authors: Amélie
authors: A
authors: Moraux
authors: Institute of Myology, Paris, France.
authors: Mélanie
authors: M
authors: Annoussamy
authors: Institute of Myology, Paris, France.
authors: Aurélie
authors: A
authors: Canal
authors: Institute of Myology, Paris, France.
authors: Valérie
authors: V
authors: Decostre
authors: Institute of Myology, Paris, France.
authors: Oumar
authors: O
authors: Diebate
authors: Institute of Myology, Paris, France; Department of Child Neurology, Groupe de Recherches sur l'Analyse Multimodale de la Fonction Cérébrale, Centre Hospitalier Universitaire d'Amiens, Amiens, France.
authors: Anne-Gaëlle
authors: AG
authors: Le Moing
authors: Institute of Myology, Paris, France.
authors: Teresa
authors: T
authors: Gidaro
authors: Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Neuromuscular Reference Center, Universitair Ziekenhuis Gent, Gent, Belgium.
authors: Nicolas
authors: N
authors: Deconinck
authors: Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Neuromuscular Reference Center, Universitair Ziekenhuis Gent, Gent, Belgium.
authors: Frauke
authors: F
authors: Van Parys
authors: Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Neuromuscular Reference Center, Universitair Ziekenhuis Gent, Gent, Belgium.
authors: Wendy
authors: W
authors: Vereecke
authors: Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Neuromuscular Reference Center, Universitair Ziekenhuis Gent, Gent, Belgium.
authors: Sylvia
authors: S
authors: Wittevrongel
authors: Department of Child Neurology, Assistance Publique-Hôpitaux de Paris Hôpital Trousseau, Paris, France.
authors: Michèle
authors: M
authors: Mayer
authors: Department of Child Neurology, Assistance Publique-Hôpitaux de Paris Hôpital Trousseau, Paris, France.
authors: Kim
authors: K
authors: Maincent
authors: Department of Child Neurology, Assistance Publique-Hôpitaux de Paris Hôpital Necker Enfants Malades, Paris, France.
authors: Isabelle
authors: I
authors: Desguerre
authors: Institute of Myology, Paris, France.
authors: Christine
authors: C
authors: Thémar-Noël
authors: Department of Child Neurology, Centre Hospitalier Régional Universitaire de Lille-Hôpital Roger-Salengro, Lille, France.
authors: Jean-Marie
authors: JM
authors: Cuisset
authors: Department of Physical Medicine and Rehabilitation, Centre Hospitalier Régional Universitaire de Lille-Hopital Pierre Swynghedauw, Lille, France.
authors: Vincent
authors: V
authors: Tiffreau
authors: Reference Center for Neuromuscular Disease, Centre hospitalier régional de la Citadelle, Liège, Belgium.
authors: Severine
authors: S
authors: Denis
authors: Reference Center for Neuromuscular Disease, Centre hospitalier régional de la Citadelle, Liège, Belgium.
authors: Virginie
authors: V
authors: Jousten
authors: Department of Pediatrics, Centre de références Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye, Université de Versailles Saint-Quentin-en-Yvelines Assistance Publique-Hôpitaux de Paris Hôpital Raymond Poincaré, Garches, France.
authors: Susana
authors: S
authors: Quijano-Roy
authors: Institute of Myology, Paris, France; Thérapie des maladies du muscle strié / Institut de Myologie, Unité Mixte de Recherche S 974 Université Pierre et Marie Curie-Institut national de la santé et de la recherche médicale-Formation de Recherche en Evolution 3617 Centre national de la recherche scientifique-Association Institut de Myologie, Paris, France.
authors: Thomas
authors: T
authors: Voit
authors: Institute of Myology, Paris, France.
authors: Jean-Yves
authors: JY
authors: Hogrel
authors: Institute of Myology, Paris, France; Reference Center for Neuromuscular Disease, Centre hospitalier régional de la Citadelle, Liège, Belgium.
authors: Laurent
authors: L
authors: Servais
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D053580:Muscle Strength / Q000187:drug effects; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy / Q000503:physiopathology*; D020127:Recovery of Function / Q000187:drug effects; D034941:Upper Extremity / Q000503:physiopathology*; D055815:Young Adult
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