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Neurorehabilitation and Neural Repair
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Clinical Trials in Spinal Muscular Atrophy: Protocol Development and Reliability of Quantitative Strength Assessment Method

Lisa Barker

Children's Hospital Medical Center, Elland and Bethesda Avenues, Cincinnati, OH 45229

Cindy Smith

Texas Scottish Rite Hospital, 2222 Welborn Street, P.O. Box 19567, Dallas, TX 75219

Betsy Perkins

Newington Children's Hospital, 181 E. Cedar Street, Newington, CT 06111

Karen Burhans

Children's Hospital Medical Center, Elland and Bethesda Avenues, Cincinnati, OH 45229

Rhonda Newman

Texas Scottish Rite Hospital, 2222 Welborn Street, P.O. Box 19567, Dallas, TX 75219

Laurie Zimmerman

Newington Children's Hospital, 181 E. Cedar Street, Newington, CT 06111

Jay Cook

Texas Scottish Rite Hospital, 2222 Welborn Street, P.O. Box 19567, Dallas, TX 75219

Barry S. Russman

Newington Children's Hospital, 181 E. Cedar Street, Newington, CT 06111

Frederick Samaha

University of Cincinnati Medical Center, Cincinnati, OH 45267

Susan Iannaccone

Texas Scottish Rite Hospital, 2222 Welborn Street, P.O. Box 19567, Dallas, TX 75219

C. Ralph Buncher

University of Cincinnati Medical Center, Cincinnati, OH 45267

Michael White

University of Cincinnati Medical Center, Cincinnati, OH 45267

Richard Browne

Texas Scottish Rite Hospital, 2222 Welborn Street, P.O. Box 19567, Dallas, TX 75219

We have developed a protocol to evaluate, prospectively, patients with spinal muscular atrophy (SMA) so that the natural course may be defined and treatment efficacy measured. A fixed myometry system with a sensitivity of 0.5 kgs. of force was used to eliminate the subjectivity experienced with manual muscle testing and handheld dynamometry. Thirteen patients were evaluated weekly for twelve weeks by the clinical evaluators (CEs). Intrarater reliability was established by demonstrating that repeated measurements by the same examiner were consistent. The CEs examined patients at each of the participating institutions on three separate occasions. Interrater reliability was demonstrated by showing that the strength evaluations made on the same subject by different evaluators did not differ significantly. The fixed myometry system was found to be a highly reliable method for evaluating very weak muscle groups in patients with SMA.

Key Words: Reliability • Quantitative muscle strength assessment • Spinal muscular atrophy.

Neurorehabilitation and Neural Repair, Vol. 6, No. 4, 175-183 (1992)
DOI: 10.1177/136140969200600402
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