title: Spirometry is affected by intelligence and behavior in Duchenne muscular dystrophy.
abstract: Children with Duchenne muscular dystrophy (DMD) have progressive respiratory muscle weakness. Spirometry monitors progress, but is effort-dependent. Intelligence quotients (IQ) average one standard deviation below normal, and behavioral disturbance is common. Our aim was to assess if impaired intelligence or behavior influences spirometry in children with DMD, and if computerized visual incentives (CVI) are beneficial. Forty-seven boys with DMD, of mean age 12.6 years (range, 6-19), were recruited. Full-scale, performance, and verbal IQ, and parent-and-teacher-reported oppositional behavior scores, were recorded. Each was divided into moderate, mild, and no impairment groups. A randomized crossover design was applied to performing spirometry with or without CVI first. A Jaeger Masterscope (version 4.60) was used. Linear regression defined the relationship between spirometry and both IQ and behavior scores. The paired Student's t- test compared spirometry performed with and without CVI for the overall group and subgroups. Boys with DMD can adequately perform spirometry. There is an association between %FEV1 and %FVC with full-scale (r = 0.50, P = 0.002; r = 0.49, P = 0.003, respectively), performance (r = 0.68, P < 0.0005; r = 0.68, P < 0.0005, respectively) and verbal (r = 0.39, P = 0.043; r = 0.36, P = 0.037, respectively) IQ, but not with parent (P = 0.77, P = 0.70, respectively) or teacher (P = 0.90, P = 0.90, respectively)-reported oppositional behavior scores. The effect of CVI was significant in those with moderate full-scale (P = 0.03), performance (P = 0.002), and verbal (P = 0.02) intellectual impairment, and moderately severe teacher-reported oppositional behavior (P = 0.02). In conclusion, spirometry results are related to intelligence in DMD. Using CVI improves the FVC obtained in those with moderate intellectual or behavioral impairment.
authors.affiliation: Department of Respiratory Medicine, Sydney Children's Hospital, Sydney, New South Wales, Australia. leanneg3@chw.edu.au
authors.forename: Leanne M
authors.initials: LM
authors.lastname: Gauld
authors.forename: Alison
authors.initials: A
authors.lastname: Boynton
authors.forename: Grant A
authors.initials: GA
authors.lastname: Betts
authors.forename: Heather
authors.initials: H
authors.lastname: Johnston
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002653:Child Behavior Disorders / Q000503:physiopathology*; D018592:Cross-Over Studies; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D007360:Intelligence / Q000502:physiology*; D007361:Intelligence Tests; D016014:Linear Models; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D011446:Prospective Studies; D012984:Software; D013147:Spirometry / Q000379:methods*; D014797:Vital Capacity / Q000502:physiology