Validation of the Childrenʼs Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND)

Department of Physical Therapy, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
Pediatric physical therapy: the official publication of the Section on Pediatrics of the American Physical Therapy Association (Impact Factor: 1.04). 12/2011; 23(4):322-6. DOI: 10.1097/PEP.0b013e3182351f04
Source: PubMed


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

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