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Prospective Analysis of Strength in Spinal Muscular Atrophy

Department of Neurology, University of Texas Southwestern Medical Center and Texas Scottish Rite Hospital for Children Dallas, TX, susani{at}ix.netcom.com

Barry S. Russman, MD

Department of Neurology, Shriner's Hospital Portland, OR

Richard H. Browne, PhD

Department of Research, Texas Scottish Rite Hospital for Children, Dallas, TX

C. Ralph Buncher, PhD

Department of Epidemiology University of Cincinnati, Cincinnati, OH

Michael White, MS

Department of Epidemiology University of Cincinnati, Cincinnati, OH

Frederick J. Samaha, MD

Department of Neurology University of Cincinnati, Cincinnati, OH

Spinal muscular atrophy is a genetic disorder of the motor neurons that causes profound hypotonia, severe weakness, and often fatal restrictive lung disease. Patients with spinal muscular atrophy present a spectrum of disease from the most severe infantile-onset type, called Werdnig-Hoffmann disease (type 1), associated with a mortality rate of up to 90%, to a late-onset mild form (type 3), wherein patients remain independently ambulatory throughout adult life. Although many clinicians agree that patients with spinal muscular atrophy lose motor abilities with age, it is unknown whether progressive weakness occurs in all patients with spinal muscular atrophy. We present here results of the first prospective study of muscle strength in patients with spinal muscular atrophy. There was no loss in muscle strength as determined by a quantitative muscle test during the observation period. However, motor function diminished dramatically in some patients with spinal muscular atrophy. Explanations for this loss of function could not be determined from our data. Decrease in motor function could be caused by factors other than loss of strength. Therefore, it is not clear from our results whether spinal muscular atrophy is a neurodegenerative disease. We conclude that treatment trials in spinal muscular atrophy should be designed with consideration of the natural history of strength and motor function in this disorder. (J Child Neurol 2000;15:97-101).

Journal of Child Neurology, Vol. 15, No. 2, 97-101 (2000)
DOI: 10.1177/088307380001500207


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