Assessment of disease progression in dysferlinopathy: A 1-year cohort study

ObjectiveTo assess the ability of functional measures to detect disease progression in dysferlinopathy over 6 months and 1 year.MethodsOne hundred ninety-three patients with dysferlinopathy were recruited to the Jain Foundation's International Clinical Outcome Study for Dysferlinopathy. Baseline, 6-month, and 1-year assessments included adapted North Star Ambulatory Assessment (a-NSAA), Motor Function Measure (MFM-20), timed function tests, 6-minute walk test (6MWT), Brooke scale, Jebsen test, manual muscle testing, and hand-held dynamometry. Patients also completed the ACTIVLIM questionnaire. Change in each measure over 6 months and 1 year was calculated and compared between disease severity (ambulant [mild, moderate, or severe based on a-NSAA score] or nonambulant [unable to complete a 10-meter walk]) and clinical diagnosis.ResultsThe functional a-NSAA test was the most sensitive to deterioration for ambulant patients overall. The a-NSAA score was the most sensitive test in the mild and moderate groups, while the 6MWT was most sensitive in the severe group. The 10-meter walk test was the only test showing significant change across all ambulant severity groups. In nonambulant patients, the MFM domain 3, wrist flexion strength, and pinch grip were most sensitive. Progression rates did not differ by clinical diagnosis. Power calculations determined that 46 moderately affected patients are required to determine clinical effectiveness for a hypothetical 1-year clinical trial based on the a-NSAA as a clinical endpoint.ConclusionCertain functional outcome measures can detect changes over 6 months and 1 year in dysferlinopathy and potentially be useful in monitoring progression in clinical trials.ClinicalTrials.gov identifier:NCT01676077

Three-antenna characterization techniques employing spherical near-field scanning with higher-order probe correction

The three-antenna technique is typically used in the context of absolute-gain measurements where no gain standard is required. When implemented in a spherical near-field test range, the conventional approach is to use near-field-to-far-field transformation algorithms with first-order probe correction, which severely limits the choice of antennas that can occupy the probe position. Two new techniques, which are based on higher-order probe correction, are presented. These enable the full characterization of up to three higher-order antennas. The first technique, where only two of the antennas need to occupy the probe position, is useful for the accurate characterization of at least the antenna that is not employed as a probe. The second technique, where all antennas, in turn, occupy the probe position, allows for the accurate characterization of each antenna.http://ieeexplore.ieee.org/xpl/RecentIssue.jsp/?punumber=8hj2023Electrical, Electronic and Computer EngineeringNon

Accurate gain measurements for large antennas using modified gain-transfer method

The classic gain-transfer method assumes a uniform plane wave incident over the apertures of the gain-transfer standard (typically a standard-gain horn) as well as the antenna under test. Variations in the incident field over the quiet zone of an antenna test range can produce large errors in the estimated gain of the antenna under test. These errors can be significant, especially when there is a large difference in aperture dimensions between the antenna under test and the standard-gain horn. In this letter, a modified gain-transfer method is described to reduce errors in gain measurements when antennas significantly larger than the standard- gain horn are measured in a test range using the gain-transfer method. The aperture of the antenna under test is usually much larger than that of the standard-gain horn, and therefore these antennas will typically be exposed to different variations in the incident field. Measured results are presented to illustrate accurate gain measurements for antennas significantly larger than the standard- gain horn.http://ieeexplore.ieee.org/xpl/RecentIssue.jsp?reload=true&punumber=7727hj201