Development of the Performance of the Upper Limb module for Duchenne muscular dystrophy

Dev Med Child Neurol. 2013 Nov;55(11):1038-45. doi: 10.1111/dmcn.12213. Epub 2013 Aug 1.

Abstract

Aim: An international Clinical Outcomes Group consisting of clinicians, scientists, patient advocacy groups, and industries identified a need for a scale to measure motor performance of the upper limb. We report the steps leading to the development of the Performance of the Upper Limb (PUL), a tool specifically designed for assessing upper limb function in ambulant and non-ambulant patients with Duchenne muscular dystrophy (DMD).

Method: The development of the PUL followed a number of steps, from the systematic review and a preliminary study exploring the suitability of the existing measures, to the application of a pilot version in a multicentric setting, with Rasch analysis of the preliminary results, leading to a revised pro forma.

Results: The PUL was specifically designed for DMD, with a conceptual framework reflecting the progression of weakness and natural history of functional decline in DMD. Modern psychometric methods were used to create a scale with robust internal reliability, validity, and hierarchical scalability; males with DMD and their families were involved iteratively throughout the process of the clinician-reported outcome assessment tool development to establish clinical meaningfulness and relevance of individual PUL items to activities of daily living.

Interpretation: The module was developed using innovative approaches and will be useful for designing clinical trials.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Activities of Daily Living
  • Disability Evaluation*
  • Humans
  • Movement Disorders / diagnosis*
  • Movement Disorders / etiology
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / pathology*
  • Psychometrics*
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Upper Extremity / physiopathology*
  • Walking