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Revised upper limb module for spinal muscular atrophy: Development of a new module
Author(s) -
Mazzone Elena S.,
Mayhew Anna,
Montes Jacqueline,
Ramsey Danielle,
Fanelli Lavinia,
Young Sally Dunaway,
Salazar Rachel,
De Sanctis Roberto,
Pasternak Amy,
Glanzman Allan,
Coratti Giorgia,
Civitello Matthew,
Forciicola,
Gee Richard,
Duong Tina,
Pane Marika,
Scoto Mariacristina,
Pera Maria Carmela,
Messina Sonia,
Tennekoon Gihan,
Day John W.,
Darras Basil T.,
Vivo Darryl C.,
Finkel Richard,
Muntoni Francesco,
Mercuri Eugenio
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25430
Subject(s) - sma* , spinal muscular atrophy , physical medicine and rehabilitation , upper limb , medicine , motor function , physical therapy , population , computer science , environmental health , algorithm
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