University of Hertfordshire

From the same journal

By the same authors

  • Elena S Mazzone
  • Anna Mayhew
  • Jacqueline Montes
  • Lavinia Fanelli
  • Sally Dunaway Young
  • Rachel Salazar
  • Roberto De Sanctis
  • Amy Pasternak
  • Allan Glanzman
  • Giorgia Coratti
  • Matthew Civitello
  • Nicola Forcina
  • Richard Gee
  • Tina Duong
  • Marika Pane
  • Mariacristina Scoto
  • Maria Carmela Pera
  • Sonia Messina
  • Gihan Tennekoon
  • John W Day
  • Basil T Darras
  • Darryl C De Vivo
  • Richard Finkel
  • Francesco Muntoni
  • Eugenio Mercuri
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Original languageEnglish
Number of pages6
Pages (from-to)869-874
JournalMuscle & nerve
Journal publication date22 May 2017
Volume55
Issue6
Early online date6 Feb 2017
DOIs
Publication statusPublished - 22 May 2017

Abstract

INTRODUCTION: There is a growing need for a robust clinical measure to assess upper limb motor function in spinal muscular atrophy (SMA), as the available scales lack sensitivity at the extremes of the clinical spectrum. We report the development of the Revised Upper Limb Module (RULM), an assessment specifically designed for upper limb function in SMA patients.

METHODS: An international panel with specific neuromuscular expertise performed a thorough review of scales currently available to assess upper limb function in SMA. This review facilitated a revision of the existing upper limb function scales to make a more robust clinical scale.

RESULTS: Multiple revisions of the scale included statistical analysis and captured clinically relevant changes to fulfill requirements by regulators and advocacy groups.

CONCLUSIONS: The resulting RULM scale shows good reliability and validity, making it a suitable tool to assess upper extremity function in the SMA population for multi-center clinical research. Muscle Nerve, 2016.

ID: 11163367