Motor Function Test Reliability During the NeuroNEXT Spinal Muscular Atrophy Infant Biomarker Study.

BACKGROUND: The NeuroNEXT SMA Infant Biomarker Study, a two year, longitudinal, multi-center study of infants with SMA type 1 and healthy infants, presented a unique opportunity to assess multi-site rater reliability on three infant motor function tests (MFTs) commonly used to assess infants with SMA type 1. OBJECTIVE: To determine the effect of prospective MFT rater training and the effect of rater experience on inter-rater and intra-rater reliability for the Test of Infant Motor Performance Screening Items (TIMPSI), the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) and the Alberta Infant Motor Scale (AIMS). METHODS: Training was conducted utilizing a novel set of motor function test (MFT) videos to optimize accurate MFT administration and reliability for the study duration. Inter- and intra-rater reliability of scoring for the TIMPSI and inter-rater reliability of scoring for the CHOP INTEND and the AIMS was assessed using intraclass correlation coefficients (ICC). Effect of rater experience on reliability was examined using ICC. Agreement with 'expert' consensus scores was examined using Pearson's correlation coefficients. RESULTS: Inter-rater reliability on all MFTs was good to excellent. Intra-rater reliability for the primary MFT, the TIMPSI, was excellent for the study duration. Agreement with 'expert' consensus was within predetermined limits (≥85%) after training. Evaluator experience with SMA and MFTs did not affect reliability. CONCLUSIONS: Reliability of scores across evaluators was demonstrated for all three study MFTs and scores were reproducible on repeated administration. Evaluator experience had no effect on reliability.

What to measure when determining orthotic needs in children with Down syndrome: a pilot study.

PURPOSE: To compare the effects of off-the-shelf foot orthoses and supramalleolar orthoses on the gait of children with Down syndrome (DS), and establish criteria for determining orthoses prescription for a child with DS. METHODS: We assessed the gait of 6 children (aged 4-7 years) with DS using the GAITRite system, and obtained height, weight, leg length, hypermobility, calcaneal eversion, navicular drop, and tibial torsion measurements. RESULTS: Supramalleolar orthoses lead to a longer cycle time than foot orthoses (P = .05) and barefoot walking (P = .03) and a lower cadence than barefoot walking (P = .04). Significant strong correlations with gait parameters were obtained for height, leg length, and hypermobility. Biomechanical measurements showed no significant correlations with gait parameters. CONCLUSIONS: The role of physical examination data, including anthropometric and biomechanical measurements in the prescription of orthoses requires further investigation.