The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): Test development and reliability

Glanzman, A.M.; Mazzone, E.; Main, M.; Pelliccioni, M.; Wood, J.; Swoboda, K.J.; Scott, C.; Pane, M.; Messina, S.; Bertini, E.; Mercuri, E.; Finkel, R.S.

doi:10.1016/j.nmd.2009.11.014

« Previous Next »Neuromuscular Disorders
Volume 20, Issue 3 , Pages 155-161, March 2010

The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): Test development and reliability

A.M. Glanzman
- Department of Physical Therapy, Children’s Hospital of Philadelphia, 3405 Civic Center Blvd., Philadelphia, PA 19104, USA
- Corresponding author. Tel.: +1 610 892 7768; fax: +1 215590 9162.
E. Mazzone
- Department of Paediatric Neurology, Catholic University, Rome, Italy
M. Main
- Dubowitz Neuromuscular Centre, Great Ormond Street Children’s Hospital, London, UK
M. Pelliccioni
- Department of Paediatric Neurology, Catholic University, Rome, Italy
J. Wood
- Primary Children’s Medical Center, Salt Lake City, UT, USA
K.J. Swoboda
- Departments of Neurology and Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA
C. Scott
- CBS Squared Inc., Fort Washington, PA, USA
M. Pane
- Department of Paediatric Neurology, Catholic University, Rome, Italy
S. Messina
- Department of Paediatric Neurology, Catholic University, Rome, Italy
- Department of Neuroscience, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy
E. Bertini
- Department of Laboratory Medicine, Unit of Molecular Medicine, Bambino Gesù Hospital, Rome, Italy
E. Mercuri
- Department of Paediatric Neurology, Catholic University, Rome, Italy
- Dubowitz Neuromuscular Centre, University College London, UK
R.S. Finkel
- Departments of Neurology and Pediatrics, Children’s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, PA, USA

Received 21 July 2009; received in revised form 16 November 2009; accepted 27 November 2009.

Abstract

The motor skills of patients with spinal muscular atrophy, type I (SMA-I) are very limited. It is difficult to quantify the motor abilities of these patients and as a result there is currently no validated measure of motor function that can be utilized as an outcome measure in clinical trials of SMA-I. We have developed the Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (“CHOP INTEND”) to evaluate the motor skills of patients with SMA-I. The test was developed following the evaluation of 26 infants with SMA-I mean age 11.5months (1.4–37.9months) with the Test of Infant Motor Performance and The Children’s Hospital of Philadelphia Test of Strength in SMA, a newly devised motor assessment for SMA. Items for the CHOP INTEND were selected by an expert panel based on item mean and standard deviation, item frequency distribution, and Chronbach’s alpha. Intra-rater reliability of the resulting test was established by test–retest of 9 infants with SMA-I over a 2month period; Intraclass correlation coefficient (ICC) (3,1)=0.96. Interrater reliability was by video analysis of a mixed group of infants with neuromuscular disease by 4 evaluators; ICC (3,4)=0.98 and in a group of 8 typically developing infants by 5 evaluators ICC (3,5)=0.93. The face validity of the CHOP INTEND is supported by the use of an expert panel in item selection; however, further validation is needed. The CHOP INTEND is a reliable measure of motor skills in patients with SMA-I and neuromuscular disorders presenting in infancy.