Pediatric Physical Therapy

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Pediatric Physical Therapy:
doi: 10.1097/PEP.0b013e3182351f04
Research Articles

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

Glanzman, Allan M. PT, DPT, PCS; McDermott, Michael P. PhD; Montes, Jacqueline PT, MA, NCS; Martens, William B. BA; Flickinger, Jean PT, MPT, PCS; Riley, Susan PT, MS, DPT, PCS; Quigley, Janet PT, DPT; Dunaway, Sally PT, DPT; O'Hagen, Jessica PT, DPT; Deng, Liyong MD; Chung, Wendy K. MD, PhD; Tawil, Rabi MD; Darras, Basil T. MD; Yang, Michele MD; Sproule, Douglas MD; De Vivo, Darryl C. MD; Kaufmann, Petra MD, MSc; Finkel, Richard S. MD; The Pediatric Neuromuscular Clinical Research Network for Spinal Muscular Atrophy (PNCR); The Muscle Study Group (MSG)

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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.

© 2011 Lippincott Williams & Wilkins, Inc.

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