Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

Pediatr Phys Ther. Winter 2011;23(4):322-6. doi: 10.1097/PEP.0b013e3182351f04.

Abstract

Purpose: Preliminary validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) for motor skill assessment in spinal muscular atrophy type I.

Methods: A total of 27 subjects 3 to 260 months old (mean = 49, SD = 69) with spinal muscular atrophy-I were evaluated with the CHOP INTEND. Subjects were evaluated as part of a multicenter natural history study.

Results: CHOP INTEND scores and age were significantly correlated (r = -0.51, P = .007; 2 survival of the motor neuron [SMN] 2 gene copies, n = 16, r = -0.60, 3 SMN2 gene copies, n = 9, r = -0.83). Respiratory support and CHOP INTEND scores were correlated (r = -0.74, P < .0001, n = 26). The CHOP INTEND and age regression in patients with 2 copies versus 3 copies of SMN2 approached significance (P = .0711, n = 25). Subjects who required respiratory support scored significantly lower (mean = 15.5, SD = 10.2 vs mean = 31.2, SD = 4.2, P < .0001, n = 27). Correlation with motor unit number estimation and combined motor unit activation were not significant.

Conclusion: The CHOP INTEND reflects measures of disease severity and supports continued exploration of the CHOP INTEND.

Publication types

  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Validation Study

MeSH terms

  • Adolescent
  • Age Factors
  • Child
  • Child Development / physiology*
  • Child, Preschool
  • Cross-Sectional Studies
  • Disability Evaluation
  • Female
  • Health Status Indicators
  • Humans
  • Infant
  • Male
  • Motor Skills / physiology*
  • Philadelphia
  • Reproducibility of Results
  • Severity of Illness Index
  • Spinal Muscular Atrophies of Childhood / diagnosis*
  • Spinal Muscular Atrophies of Childhood / pathology
  • Statistics as Topic
  • Young Adult