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The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): test development and
A M Glanzman1, E Mazzone, M Main
1Department of Physical Therapy, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. glanzmana@email.chop.edu
Insights
The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) reliably measures motor skills in infants with spinal muscular atrophy type I (SMA-I). This new tool is crucial for clinical trials evaluating SMA-I treatments.
Area of Science:
- Pediatric Neurology
- Neuromuscular Disorders
- Clinical Outcome Measures
Background:
- Spinal muscular atrophy type I (SMA-I) severely limits infant motor skills.
- Quantifying motor abilities in SMA-I is challenging, lacking validated clinical trial outcome measures.
- Existing assessments do not adequately capture the specific motor deficits in SMA-I.
Purpose of the Study:
- To develop and validate a reliable motor function assessment for infants with SMA-I.
- To introduce the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) as a clinical outcome measure.
- To establish the psychometric properties of the CHOP INTEND for use in research and clinical settings.
Main Methods:
- The CHOP INTEND was developed using data from 26 infants with SMA-I, incorporating items from existing tests and expert evaluation.
- Item selection for the CHOP INTEND was guided by statistical analysis (mean, standard deviation, frequency distribution, Chronbach's alpha).
- Reliability was assessed through intra-rater (test-retest) and inter-rater (video analysis) analyses in infants with and without neuromuscular disease.
Main Results:
- The CHOP INTEND demonstrated high intra-rater reliability (ICC=0.96) and inter-rater reliability (ICC=0.98 and 0.93).
- Expert panel input supported the face validity of the CHOP INTEND.
- The test effectively evaluates motor skills in infants with SMA-I and other infantile neuromuscular disorders.
Conclusions:
- The CHOP INTEND is a reliable and valid measure for assessing motor function in infants with SMA-I.
- This tool addresses the critical need for a validated outcome measure in SMA-I clinical trials.
- Further validation studies are recommended to expand the utility of the CHOP INTEND.
Abstract:
The motor skills of patients with spinal muscular atrophy, type I (SMA-I) are very limited. It is difficult to quantify the motor abilities of these patients and as a result there is currently no validated measure of motor function that can be utilized as an outcome measure in clinical trials of SMA-I. We have developed the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders ("CHOP INTEND") to evaluate the motor skills of patients with SMA-I. The test was developed following the evaluation of 26 infants with SMA-I mean age 11.5 months (1.4-37.9 months) with the Test of Infant Motor Performance and The Children's Hospital of Philadelphia Test of Strength in SMA, a newly devised motor assessment for SMA. Items for the CHOP INTEND were selected by an expert panel based on item mean and standard deviation, item frequency distribution, and Chronbach's alpha. Intra-rater reliability of the resulting test was established by test-retest of 9 infants with SMA-I over a 2 month period; Intraclass correlation coefficient (ICC) (3,1)=0.96. Interrater reliability was by video analysis of a mixed group of infants with neuromuscular disease by 4 evaluators; ICC (3,4)=0.98 and in a group of 8 typically developing infants by 5 evaluators ICC (3,5)=0.93. The face validity of the CHOP INTEND is supported by the use of an expert panel in item selection; however, further validation is needed. The CHOP INTEND is a reliable measure of motor skills in patients with SMA-I and neuromuscular disorders presenting in infancy.
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