Motor proficiency in young children with Prader-Willi syndrome: a preliminary report

Abstract

BACKGROUND: Systematic documentation of motor characteristics in young children with Prader-Willi syndrome (PWS) is vital as access to treatments improves. AIM: To characterize motor proficiency (MP) in young children with PWS. METHOD: Participants included 6 children (3 male and 3 female) with PWS and 13 children with neurotypical development (NT), (9 male and 4 female) ages 4-6 years. Five out of six children with PWS had been on growth hormone replacement therapy (GHRT) for >3 years. Some children with PWS exhibited cognitive delays and others performed within the average range (Intellectual quotient mean± standard deviation = 65.3 ± 7.62, range = 47 – 94). MP was measured using the Short Form of the Bruininks-Oseretsky Test of Motor Proficiency-Second Edition (BOT-2-SF). RESULTS: Children with PWS scored lower than children with NT in all areas of MP except for fine motor integration. All children with PWS scored well-below average for total MP; children with NT scored average (n=10) or above-average (n=3) for total MP, respectively. CONCLUSION: At this young age children with PWS universally exhibited poor MP despite most of them being on GHRT and some exhibited intellectual functioning in the average range. Evidence of BOT-2-SF floor effects underscores the need to refine assessment procedures and enhance measurement precision for this population.