{
  "abstract": "Background Facioscapulohumeral Dystrophy (FSHD) is an autosomal dominant, progressive muscle disease, leading to chronic disability through loss of muscle and strength. Monitoring muscle strength is an essential outcome. Methods such as manual muscle testing (MMT) lack precision at an individual muscle level, and fixed frame quantitative muscle assessments are resource intensive. Objective muscle strength assessment using handheld dynamometer (HHD) may offer an objective alternative to these methods. We appraise the reliability, and validity of HHD in FSHD as an in-clinic assessment.Methods A standardised protocol of assessments using FSHD Clinical Score (FCS), MMT, MicroFET2 HHD and Biodex dynamometer. Test-retest reliability of HHD was assessed through repeated assessments.Results HHD assessments were feasible and well tolerated. Test-retest reliability was excellent for all movements assessed (ICC >0.95). ICC was good to excellent between HHD and Biodex for elbow flexion (0.89), knee flexion (0.87) and ankle dorsiflexion (0.96). MMT demonstrated substantial ceiling effects as compared with HHD. Strong correlation was found between disease severity (FCS) and HHD score (ρ = 0.74).Conclusion HHD is a feasible, reliable, and valid method of strength assessment in FSHD, when used with a standardised protocol, in appropriate muscle groups, by a trained evaluator.j.street1@nhs.net",
  "authors": [
    {
      "affiliations": [
        "Academic Unit of Neuromuscular Disease, Sheffield Teaching Hospitals",
        "SITraN, University of Sheffield"
      ],
      "name": "Street Jon"
    },
    {
      "affiliations": [
        "INSIGNEO, University of Sheffield"
      ],
      "name": "Rowson Jennifer"
    },
    {
      "affiliations": [
        "Academic Unit of Neuromuscular Disease, Sheffield Teaching Hospitals",
        "SITraN, University of Sheffield"
      ],
      "name": "McDermott Christopher J"
    },
    {
      "affiliations": [
        "Academic Unit of Neuromuscular Disease, Sheffield Teaching Hospitals",
        "SITraN, University of Sheffield"
      ],
      "name": "Hewamadduma Channa"
    }
  ],
  "title": "151 Muscle strength assessment in clinic using handheld dynamometry in FSHD- is it feasible and valid?",
  "uid": "3cdf7a59-8f23-57da-a934-cb05bfa51141"
}