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Pulmonary Function in Spinal Muscular Atrophy

University of Cincinnati Medical Center

C.R. Buncher, PhD

University of Cincinnati Medical Center

B.S. Russman, MD

Newington Children's Hospital (Dr Russman, Ms Perkins, and Ms Zimmerman), Newington, CT

M.L. White, MS

University of Cincinnati Medical Center

S.T. Iannaccone, MD

Texas Scottish Rite Hospital (Dr Iannaccone and Ms Smith), Dallas, TX

L. Barker

Children's Hospital Medical Center (Mrs Burhans and Ms Barker), Cincinnati, OH

K. Burhans

Children's Hospital Medical Center (Mrs Burhans and Ms Barker), Cincinnati, OH

C. Smith

Texas Scottish Rite Hospital (Dr Iannaccone and Ms Smith), Dallas, TX

B. Perkins

Newington Children's Hospital (Dr Russman, Ms Perkins, and Ms Zimmerman), Newington, CT

L. Zimmerman

Newington Children's Hospital (Dr Russman, Ms Perkins, and Ms Zimmerman), Newington, CT

We present the first prospective study on pulmonary function in spinal muscular atrophy patients. Seventy-seven spinal muscular atrophy patients, ages 5 to 18 years, from three centers, were studied with regard to forced vital capacity, using height as a predictor. Patients were categorized into four motor function categories. The highest-functioning group had normal or near-normal values, and those who sat with support had the lowest values. Those with intermediate function had intermediate values. Forced vital capacity was studied longitudinally in 40 spinal muscular atrophy patients for 1.1 to 4.4 years. Eighty-eight percent of patients grew in height, but only 35% showed an increase in height-adjusted forced vital capacity percent. In those patients with the least function, 100% lost height-adjusted forced vital capacity over time. In those patients with the highest function, 57% lost height-adjusted forced vital capacity. In addition, the basic forced vital capacity, not correlated to height, decreased in 43% of cases. These pulmonary function alterations appear to be important determinants for function and survival in spinal muscular atrophy patients. (J Child Neurol 1994;9:326-329).

Journal of Child Neurology, Vol. 9, No. 3, 326-329 (1994)
DOI: 10.1177/088307389400900321


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