Journal
NEUROLOGY
Volume 75, Issue 6, Pages 526-531Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/WNL.0b013e3181eccf8f
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Funding
- Spinal Muscular Atrophy Foundation
- NIH (NIAMS) [2P01 NS040828-6A11 [co-PI]]
- SMA Foundation
- Muscular Dystrophy Association
- New England Research Institutes/SMA Foundation
- Slaney Family Fund for SMA
- NIH [NINDS R01NS055099 [PI], NINDS K24NS060951-01A1 [PI]]
- ALS Association
- Center for Integration of Medicine and Innovative Technology
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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. Neurology (R) 2010; 75: 526-531
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