Validation of the North Star Assessment for Limb-Girdle Type Muscular Dystrophies.
Meredith K James, Lindsay N Alfano, Robert Muni-Lofra and 12 others
PMID 35932452WHAT IT FOUND
The North Star Assessment for Limb-Girdle Type Muscular Dystrophies reliably measures motor function across 14 different LGMD subtypes.
It works for both ambulatory and non-ambulatory patients, with high agreement between different therapists scoring the same video.
Key findings
01The scale demonstrated high reliability and unidimensionality across a broad range of LGMD subtypes.
02Scores from different physical therapists showed excellent agreement, as did scores from the same therapist at two different times.
03The assessment successfully targeted both ambulatory and non-ambulatory individuals, covering the full spectrum of motor ability.
STILL TO COME
How it was doneWhat they foundWhat it means for PTsWhat it means for OTs
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What it does not show
The study was cross-sectional, so it did not prove that the scale can detect changes in function over time (responsiveness) in these specific subtypes, although longitudinal work is underway. The sample was a convenience sample from two highly specialized international centers, so it may not represent the full prevalence of LGMD subtypes in the general population. There was a ceiling effect for the strongest, asymptomatic individuals, meaning the scale might not capture subtle changes in those with very mild disease. Some items showed developmental differences in children under 5 years, suggesting caution when interpreting scores in very young children for items like jumping and hopping.
Declared interests
The study was funded by the Jain Foundation. The funder played no role in the design, conduct, or reporting of the study.
The easy way to misread this
Do not assume the NSAD measures upper limb function adequately. The study found that some ambulatory patients with high NSAD scores still had significant upper limb impairment, so you must use a separate tool like the PUL to assess arm strength and function.