Assessing spinal muscular atrophy with quantitative ultrasound
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Abstract
Objective: To assess the value of quantitative ultrasound in patients with type 2 and 3 spinal muscular atrophy (SMA).
Methods: Twenty-five patients with SMA (15 type 2 and 10 type 3) and 21 normal subjects were enrolled for this observational study. Strength of biceps brachii, wrist extensors, quadriceps, and tibialis anterior were measured with hand-held dynamometry. In addition, these 4 muscles were studied with a standard ultrasound system using a 5-MHz probe, and luminosity values for each muscle and the overlying subcutaneous fat were obtained by subsequent image analysis. A luminosity ratio (LR) for each muscle was calculated by dividing the muscle by the subcutaneous fat luminosity. The LR and strength scores for all 4 muscles were averaged to provide a single summary value for each patient.
Results: The LRs increased with disease severity: 1.27 ± 0.26 for normal subjects, 2.43 ± 0.78 for type 3 SMA, and 3.85 ± 1.3 for type 2 SMA (p < 0.001). Taking all the normal subject and patient data together, there was a good correlation between strength and LR (r = −0.711, p < 0.001). There was also a moderate relationship between LR and strength in the patients with SMA alone (r = −0.588, p = 0.008), and, as expected, a nonsignificant relationship between LR and strength in normal subjects (r = −0.011).
Conclusions: Quantitative ultrasound has the potential of serving as a marker of SMA severity and may be useful in future clinical trials.
Footnotes
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Study funding: Supported by the Spinal Muscular Atrophy Foundation.
Disclosure: Author disclosures are provided at the end of the article.
Received December 5, 2009. Accepted in final form April 26, 2010.
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Letters: Rapid online correspondence
- Assessing spinal muscular atrophy with quantitative ultrasound
- Sigrid Pillen, Department of Neurology and Clinical Neurophysiology, Radboud University Nijmegen Medical Center, KNF 920; P.O. Box 9101; 6500HB Nijmegen, The Netherlandss.pillen@cukz.umcn.nl
- Nens van Alfen (Nijmegen, The Netherlands; n.vanalfen@neuro.umcn.nl), Eric J. Sorenson (Rochester, MN; Sorenson.Eric@mayo.edu), Andrea J. Boon (Rochester, MN; Boon.Andrea@mayo.edu)
Submitted October 20, 2010 - Reply from the authors
- Jim S. Wu, Beth Israel Deaconess Medical Center, 330 Brookline Ave Boston MA 02215jswu@bidmc.harvard.edu
- Basil T. Darras (Boston, MA; Basil.Darras@childrens.harvard.edu), Seward B. Rutkove (Boston, MA; srutkove@bidmc.harvard.edu)
Submitted October 20, 2010
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