title: Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review.
abstract: OBJECTIVE
  The aims of this review were (1) to provide insight into the natural course of upper-extremity (UE) impairments and UE activity limitations associated with facioscapulohumeral dystrophy (FSHD) and limb-girdle muscular dystrophies (LGMD), and (2) to provide an overview of outcome measures used to evaluate UE function and activity in patients with FSHD and LGMD.
  
  
  METHODS
  Scientific literature databases (PubMed, MEDLINE, EMBASE, CINAHL and Cochrane) were searched for relevant publications.
  
  
  METHODS
  (1) studies that included persons with a diagnosis of FSHD or LGMD; and (2) studies that reported the natural course of the UE functions and/or activity with outcome measures at these levels.
  
  
  RESULTS
  247 publications were screened, of which 16 fulfilled the selection criteria. Most studies used manual muscle testing (MMT) to evaluate UE function and the Brooke Scale to evaluate UE mobility activities. The clinical picture of UE impairments and limitations of UE activities in FSHD and LGMD patients was highly variable. In general, FSHD and LGMD patients experience difficulty elevating their upper extremities and the execution of tasks takes considerably longer time.
  
  
  CONCLUSIONS
  The clinical course of UE impairments and activity limitations associated with FSHD and LGMD is difficult to predict due to its high variability. Although measures like MMT and the Brooke Scale are often used, there is a lack of more specific outcome measures to assess UE function and UE capacity and performance in daily life. Measures such as 3D motion analysis and electromyography (EMG) recordings are recommended to provide additional insight in UE function. Questionnaires like the Abilhand are recommended to assess UE capacity and accelerometry to assess UE performance in daily life.
  
  
  CONCLUSIONS
  There is a need for specific outcome measures on the level of UE activity. Both the level of capacity and performance should be assessed. Possible outcome measures include 3D motion analysis to assess UE function, questionnaires like the Abilhand to assess UE capacity and accelerometry to assess performance of UE activities in daily life.
authors.affiliation: Department of Rehabilitation, Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands and.
authors.forename: Arjen
authors.initials: A
authors.lastname: Bergsma
authors.forename: Edith H C
authors.initials: EH
authors.lastname: Cup
authors.forename: Alexander C H
authors.initials: AC
authors.lastname: Geurts
authors.forename: Imelda J M
authors.initials: IJ
authors.lastname: de Groot
mesh_terms: D000203:Activities of Daily Living; D004576:Electromyography; D006801:Humans; D049288:Muscular Dystrophies, Limb-Girdle / Q000503:physiopathology / Q000534:rehabilitation*; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000503:physiopathology / Q000534:rehabilitation*; D020127:Recovery of Function; D016896:Treatment Outcome; D034941:Upper Extremity / Q000503:physiopathology*
keywords: Activities of daily living; MeSH; arm; muscular-dystrophy; upper extremity