Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)
Title
Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)
Creator
Glanzman A M; McDermott M P; Montes J; Martens W B; Flickinger J; Riley S; Quigley J; Dunaway S; O'Hagen J; Deng L Y; Chung W K; Tawil R; Darras B T; Yang M; Sproule D; De Vivo D C; Kaufmann P; Finkel R S; Pediatric Neuromuscular Clinical Research; Muscle Study Group
Identifier
Publisher
Pediatric Physical Therapy
Date
2011
Subject
infant; Pediatrics; Rehabilitation; validity; outcome assessment; child; female; male; disease severity; (health care); atrophies of childhood/diagnosis; child development/physiology; childhood/physiopathology; disability evaluation; humans; motor skills/physiology; natural-history; physical therapy/methods; preschool; psychometrics/methods; severity of illness index; sma; smn2 copy number; spinal muscular; spinal muscular atrophies of; spinal muscular-atrophy; tone and motor problems; SMA1; tool development; scale development; CHOP INTEND
Description
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. (Pediatr Phys Ther 2011; 23: 322-326)
Rights
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URL Address
Citation
Glanzman A M; McDermott M P; Montes J; Martens W B; Flickinger J; Riley S; Quigley J; Dunaway S; O'Hagen J; Deng L Y; Chung W K; Tawil R; Darras B T; Yang M; Sproule D; De Vivo D C; Kaufmann P; Finkel R S; Pediatric Neuromuscular Clinical Research; Muscle Study Group, “Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND),” Pediatric Palliative Care Library, accessed February 10, 2025, https://pedpalascnetlibrary.omeka.net/items/show/16666.