Digital stereophotogrammetry as a new technique to quantify truncal deformity: a pilot study in persons with osteogenesis imperfecta.

The objective of this pilot study was to determine the usability of stereophotogrammetry (SP) as a noninvasive technique for obtaining linear measures and anatomical data of the torso in people with osteogenesis imperfecta in comparison with clinical observations. Ten participants were recruited from subjects enrolled in ongoing institutional review board-approved osteogenesis imperfecta protocols at the National Institute of Child Health and Human Development. Using a Gulick tape measure, anthropometer, and the SP system proprietary software, linear measurements of the torso were taken. In addition, the presence or absence of specific torso deformities was documented from both clinical observation and evaluation of SP images. Measurements of torso diameter and circumference by SP demonstrated strong agreement with the manual measurements (intraclass correlation coefficient = 0.995 and 0.964, respectively). Substantial and statistically significant agreement was present between SP image evaluation and clinical observation for pectus carinatum (κ = 0.52 ± 0.23) and thoracic scoliosis (κ = 0.72 ± 0.12). The kappa values between clinical observation and SP evaluations of other torso deformities were not significant. The strong correlations and P values determined by this study demonstrate the potential value of SP in studying persons with truncal deformities. However, the weak agreement between SP and some clinical observations suggests that further development of SP image analysis tools is required before SP can be used as a standard method of diagnosis or assessment of treatment success.

Brief assessment of motor function: content validity and reliability of the upper extremity gross motor scale.

Content validity and reliability of the Brief Assessment of Motor Function (BAMF) Upper Extremity Gross Motor Scale (UEGMS) were evaluated in this prospective, descriptive study. The UEGMS is one of five BAMF ordinal scales designed for quick documentation of gross, fine, and oral motor skill levels. Designed to be independent of age and diagnosis, it is intended for use for infants through young adults. An expert panel of 17 physical therapists and 13 occupational therapists refined the content by responding to a standard questionnaire comprised of questions, which asked whether each item should be included, is clearly worded, should be reordered higher or lower, is functionally relevant, and is easily discriminated. Ratings of content validity exceeded the criterion except for two items, which may represent different perspectives of physical and occupational therapists. The UEGMS was modified using the quantitative and qualitative feedback from the questionnaires. For reliability, five raters scored videotaped motor performances of 10 children. Coefficients for inter-rater (0.94) and intra-rater (0.95) reliability were high. The results provide evidence of content validity and reliability of the UEGMS for the assessment of UEGM skill.

Brief assessment of motor function: content validity and reliability of the oral motor scales.

OBJECTIVE: The Brief Assessment of Motor Function consists of five 0- to 10-point hierarchical scales designed for rapid assessment of gross, fine, and oral motor skills. We describe the development and evaluation of the two Brief Assessment of Motor Function Oral Motor Scales: Oral Motor Articulation and Oral Motor Deglutition. DESIGN: This validation study employed an expert panel of 28 speech-language pathologists, who rated the Brief Assessment of Motor Function Oral Motor Scales items on a scale from 1 to 4 (disagree to agree) to establish content validity. For reliability, oral motor performances of 18 participants (6 mos-20 yrs) were videotaped to represent a wide range of articulation and deglutition capabilities. Four speech-language pathologists, and 1 undergraduate and 10 graduate speech-language pathology students rated the participants' taped samples using the Brief Assessment of Motor Function Oral Motor Scales. RESULTS: All items on the content validity questionnaire had average agreement scores that exceeded criteria, except two, which were not clearly worded; these were clarified. Interrater and intrarater reliability values were 0.997 and 0.986 for the Oral Motor Articulation Scale and 0.977 and 0.997 for the Oral Motor Deglutition Scale. CONCLUSIONS: Expert feedback and reliability procedures suggest that the Brief Assessment of Motor Function Oral Motor Articulation and Deglutition Scales represent the content that they are designed to assess and are reliable for rapid assessment of oral motor skills.

Brief assessment of motor function: content validity and reliability of the fine motor scale.

PURPOSE: The Brief Assessment of Motor Function Fine Motor Scale (FMS) allows rapid assessment, independent of age. This study was done to establish content validity of the FMS and to demonstrate FMS reliability. METHODS: A standard questionnaire ("Disagree" to "Agree," 1-4) was emailed to 28 expert panel members. Ten children with diagnoses including Proteus, Sheldon-Freeman, Smith-Lemli-Opitz, and Smith-Magenis syndromes were videotaped for reliability trials. RESULTS: Expert panel members agreed that all 28 items should be included (means, 3.43-3.89); were functionally relevant (means, 2.93-3.82), were clearly worded (means, 2.71-3.61), and were easily discriminated (means, 3.32-4.0). Kappa values for interrater and intrarater reliability were 0.978 and 0.993, respectively. CONCLUSIONS: Feedback from an expert Panel supported content validity of the Brief Assessment of Motor Function FMS. Kappa values for interrater and intrarater reliability suggest this is a reliable instrument for rapid, objective fine motor assessment.

Intra-rater and inter-rater reliability of the 10-point Manual Muscle Test (MMT) of strength in children with juvenile idiopathic inflammatory myopathies (JIIM).

OBJECTIVE: Children with juvenile idiopathic inflammatory myopathies (JIIM) present with muscle inflammation and decreased strength that may affect their functional abilities. The purpose of this study was to determine the intra-rater and inter-rater reliability of the 0 to 10-point manual muscle testing method for children with JIIM. METHODS: For the intra-rater and inter-rater reliability studies, 10 and 9 children with JIIM participated, respectively. For intra-rater reliability, one pediatric therapist completed two assessments in one day with a one-hour break. For inter-rater reliability, four therapists assessed the same child within a single morning. RESULTS: Spearman correlations for intra-rater reliability ranged from 0.70 to 1.00. Kendall's W coefficient for inter-rater reliability of groups of muscles (total, proximal, distal, and peripheral) ranged from 0.51 to 0.76. CONCLUSIONS: The total, proximal, and peripheral Manual Muscle Test (MMT) score, using the 0-10 point scale, has acceptable reliability in JIIM patients.

Controlled trial of pamidronate in children with types III and IV osteogenesis imperfecta confirms vertebral gains but not short-term functional improvement.

UNLABELLED: Bisphosphonates have been widely administered to children with OI based on observational trials. A randomized controlled trial of q3m intravenous pamidronate in children with types III and IV OI yielded positive vertebral changes in DXA and geometry after 1 year of treatment, but no further significant improvement during extended treatment. The treated group did not experience significantly decreased pain or long bone fractures or have increased motor function or muscle strength. INTRODUCTION: Bisphosphonates, antiresorptive drugs for osteoporosis, are widely administered to children with osteogenesis imperfecta (OI). Uncontrolled pamidronate trials in OI reported increased BMD, vertebral coronal area, and mobility, and decreased pain. We conducted a randomized controlled trial of pamidronate in children with types III and IV OI. MATERIALS AND METHODS: This randomized trial included 18 children (4-13 years of age) with types III and IV OI. The first study year was controlled; 9 children received pamidronate (10 mg/m2/day IV for 3 days every 3 months). Four children in each group also received recombinant growth hormone (rGH) injections (0.06 mg/kg/day for 6 days/week). Seven children in the treatment group received pamidronate for an additional 6-21 months. All patients had L1-L4 DXA, spine QCT, spine radiographs, and musculoskeletal and functional testing. RESULTS: In the controlled phase, treated patients experienced a significant increase in L1-L4 DXA z score (p < 0.001) and increased L1-L4 mid-vertebral height (p = 0.014) and total vertebral area (p = 0.003) compared with controls. During extended treatment, DXA z scores and vertebral heights and areas did not increase significantly beyond the 12-month values. Fracture rate decreased significantly in the upper extremities (p = 0.04) but not the lower extremities (p = 0.09) during the first year of treatment. Gross motor function, muscle strength, and pain did not change significantly during the controlled or extended treatment phases. CONCLUSIONS: A controlled trial confirmed the spine benefits of short-term pamidronate treatment in children with types III and IV OI. Pamidronate increased L1-L4 vertebral DXA and decreased vertebral compressions and upper extremity fractures. Vertebral measures did not improve during the extended treatment phase. The treatment group did not experience decreased lower extremity long bone fractures, significant improvement in growth, ambulation, muscle strength, or pain. There was substantial variability in individual response to treatment.

Ultrasound imaging distinguishes between normal and weak muscle.

OBJECTIVE: To determine whether real-time ultrasound imaging can provide quantitative data that distinguish pathologic from healthy muscle and that correlate with strength measures. DESIGN: Nonrandomized matched-pair, repeated-measures design. SETTING: Ultrasound imaging laboratory, rehabilitation medicine department, government research hospital. PARTICIPANTS: Nine patients with stable active or inactive myositis, stratified into 3 groups based on their 10-point manual muscle test (MMT) scores, and 9 age- and gender-matched controls. INTERVENTIONS: Maximal isometric contraction of the rectus femoris muscle in 2 knee-flexion positions (60 degrees, 90 degrees ) during simultaneous ultrasound imaging and muscle force dynamometry. MAIN OUTCOME MEASURES: Changes of the rectus femoris muscle in horizontal (X) and vertical (Y) diameters between relaxed and contracted states, and muscle force measurements. RESULTS: The X diameters decreased and the Y diameters increased during isometric contraction in all participants. For each group, average changes in cross-sectional diameters were consistently higher in controls than in patients. Patients with MMT less than 8 differed significantly from controls in both X and Y dimensions. A moderately strong correlation was found between muscle force and the Y diameter during contraction at 60 degrees (r =.78) and 90 degrees (r =.67) knee-flexion angles. CONCLUSIONS: Ultrasonography provided a quantitative measure of change between relaxed and contracted state of muscle, which correlated with muscle force. Ultrasound identified significant differences in cross-sectional diameters between the myopathic and normal muscles sampled and may be useful for measuring muscle response to drug and exercise therapy.

Temperament and physical performance in children with osteogenesis imperfecta.

OBJECTIVE: Children with osteogenesis imperfecta (OI) must participate in therapy to achieve motor performance objectives. Their behavioral style may influence motor performance. For this reason, the temperament of children with types III or IV OI was assessed prospectively to 1) compare their temperament with that of nondisabled children, 2) investigate the relationship between temperament and gross motor performance, and 3) examine relationships among temperament, parental overprotection and coping, physical activity, muscle strength, and motor performance. METHODS: Age-appropriate Carey Temperament Scales, Brief Assessment of Motor Function (BAMF), and the Vulnerable Child/Overprotecting Parents Scale were completed for 35 children 1 to 12 years old. Additional measures included the Childhood Health Assessment Questionnaire, Parent Daily Hassles Scale, manual muscle testing, Pediatric Activity Record, and a Summed Severity Score. Spearman correlations and multiple regression were used to identify and predict significant relationships. RESULTS: Temperament of children with OI differed from age-based norms in only 1 domain: activity. Motor performance (BAMF) correlated significantly with 3 domains of temperament: persistence (r = -.48), approach (r = -.34), and activity (r =.40). Activity was also related to the ratio of head circumference to body length (r = -.45) and the number of fractures in the preceding year (r = -.35). Parents' reports of their daily hassles significantly correlated with several domains of the child's temperament. No significant relationships were identified between parental overprotection and temperament or motor performance. CONCLUSIONS: The temperament of children with types III and IV OI does not differ from that of their nondisabled peers, with the exception of lower activity scores. Although it is considered a biological attribute, the expression of temperament, specifically activity, may be influenced by learned behaviors. Because gross motor performance is related to activity, persistence, and approach/avoidance, knowledge of an individual's temperament may enhance the child's ability to benefit from interventions to improve motor skill and activity levels.

Brief assessment of motor function: reliability and concurrent validity of the Gross Motor Scale.

OBJECTIVE: The Brief Assessment of Motor Function (BAMF) is a series of 10-point ordinal scales developed for rapid description of gross motor, fine motor, and oral motor performance. We examined interrater and intrarater reliability and concurrent validity of the BAMF Gross Motor Scale. DESIGN: This validation study included 48 children (age, 5 mo to 17 yr) with a wide range of gross motor capability. Ten children with varied diagnoses participated in the reliability study. For concurrent validity, the BAMF performance of 38 children with osteogenesis imperfecta was compared with scores on the Peabody Developmental Motor Scales, laboratory gait analysis, and manual muscle testing. RESULTS: Reliability values for intraclass correlations were 0.996 (interrater) and 1.00 (intrarater). Significant relationships were identified between the BAMF and gait speed ( r = 0.68, P < 0.0001), stride length (r = 0.71, P < 0.0001), duration of double-limb support ( r = -0.40, P < 0.03), number of weak muscles (r = -0.74, P< 0.0001), and the Peabody Developmental Motor Scales (r = 0.95, P < 0.0001). Number of weak muscles was the strongest predictor of BAMF score R2 = 0.5080, F = 24.77, P < 0.0001). CONCLUSIONS: The BAMF demonstrates good reliability for children with a range of diagnoses and acceptable concurrent validity with gross motor development, muscle strength, and formal gait assessment in children with osteogenesis imperfecta.