Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Jun;55(6):869-874.
doi: 10.1002/mus.25430. Epub 2017 Feb 6.

Revised upper limb module for spinal muscular atrophy: Development of a new module

Elena S Mazzone  1 , Anna Mayhew  2 , Jacqueline Montes  3 , Danielle Ramsey  4 , Lavinia Fanelli  1 , Sally Dunaway Young  3 , Rachel Salazar  3 , Roberto De Sanctis  1 , Amy Pasternak  5 , Allan Glanzman  6 , Giorgia Coratti  1 , Matthew Civitello  7 , Nicola Forcina  1 , Richard Gee  8 , Tina Duong  8 , Marika Pane  1 , Mariacristina Scoto  4 , Maria Carmela Pera  1 , Sonia Messina  9 , Gihan Tennekoon  6 , John W Day  8 , Basil T Darras  5 , Darryl C De Vivo  3 , Richard Finkel  7 , Francesco Muntoni  4 , Eugenio Mercuri  1
Affiliations

Revised upper limb module for spinal muscular atrophy: Development of a new module

Elena S Mazzone et al. Muscle Nerve. 2017 Jun.

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 55: 869-874, 2017.

Keywords: Hammersmith functional motor scale; Rasch analysis; functional assessment; outcome measures; spinal muscular atrophy; upper limb function.

PubMed Disclaimer

Publication types