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Rasch-analysis of Clinical Severity in FSHD

Completed
Conditions
Facioscapulohumeral Muscular Dystrophy
Registration Number
NCT02766985
Lead Sponsor
University of Kansas Medical Center
Brief Summary

The purpose of this study is to make a standardized and scalable Rasch-built clinical severity scale to help in finding genetic and environmental modifiers of disease in Facioscapulohumeral muscular dystrophy (FSHD).

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
44
Inclusion Criteria
  • Genetic confirmation of FSHD (either FSHD-1 or FSHD-2)
Exclusion Criteria
  • Do not understand English
  • Unable to provide informed consent

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
FSHD Clinical Severity Scale1 day

Measure severity of FSHD.

Secondary Outcome Measures
NameTimeMethod
Quantitative strength testing1 day
Brooks scale1 day

Measures upper extremity function.Scores on the scale range from 1-6. A score of 1 means the person can elevate their arms above their head while arms remain straight. 6 means the person cannot elevate their arms to their mouth and do not have useful function of their hands.

Vignos scale1 day

Measures lower extremity function. Scores on the scale range from 1-10. A score of 1 means the person can walk and climb stairs without assistance. A score of 10 means the person is bedridden.

Manual muscle testing1 day

Measure person's range of motion. Scored on a scale with range of 0-5. Score of 0 means no movement. Score of 5 means the person has a full range of movement.

Iowa Oral Performance Instrument (IOPI)1 day

IOPI measures lip and tongue strength by measuring the amount of pressure a person can exert on the tool.

Patient-reported outcome1 day
Functional testing1 day
Reparatory function testing1 day

Trial Locations

Locations (2)

University of Utah

🇺🇸

Salt Lake City, Utah, United States

University of Kansas Medical Center

🇺🇸

Kansas City, Kansas, United States

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