title: Validation of the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND).
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  The CHOP INTEND reflects measures of disease severity and supports continued exploration of the CHOP INTEND.
authors.affiliation: Department of Physical Therapy, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA. glanzmana@email.chop.edu
authors.forename: Allan M
authors.initials: AM
authors.lastname: Glanzman
authors.forename: Michael P
authors.initials: MP
authors.lastname: McDermott
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Montes
authors.forename: William B
authors.initials: WB
authors.lastname: Martens
authors.forename: Jean
authors.initials: J
authors.lastname: Flickinger
authors.forename: Susan
authors.initials: S
authors.lastname: Riley
authors.forename: Janet
authors.initials: J
authors.lastname: Quigley
authors.forename: Sally
authors.initials: S
authors.lastname: Dunaway
authors.forename: Jessica
authors.initials: J
authors.lastname: O'Hagen
authors.forename: Liyong
authors.initials: L
authors.lastname: Deng
authors.forename: Wendy K
authors.initials: WK
authors.lastname: Chung
authors.forename: Rabi
authors.initials: R
authors.lastname: Tawil
authors.forename: Basil T
authors.initials: BT
authors.lastname: Darras
authors.forename: Michele
authors.initials: M
authors.lastname: Yang
authors.forename: Douglas
authors.initials: D
authors.lastname: Sproule
authors.forename: Darryl C
authors.initials: DC
authors.lastname: De Vivo
authors.forename: Petra
authors.initials: P
authors.lastname: Kaufmann
authors.forename: Richard S
authors.initials: RS
authors.lastname: Finkel
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002657:Child Development / Q000502:physiology*; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004185:Disability Evaluation; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D007223:Infant; D008297:Male; D009048:Motor Skills / Q000502:physiology*; D015143:Philadelphia; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis* / Q000473:pathology; D013223:Statistics as Topic; D055815:Young Adult