A spatio-temporal and kinematic description of self-selected walking in adults with Achondroplasia.

BACKGROUND: Achondroplasia is characterised by a shorter appendicular limb to torso ratio, compared to age matched individuals of average stature (controls). Despite the well documented shorter leg length of individuals with compared to controls, there are few complete descriptions of gait kinematics reported for the population. AIM: The aim of this study was to report the spatio-temporal and kinematic characteristics of self-selected walking (SSW) in a group with Achondroplasia (N = 10) and age matched group without Achondroplasia (controls, N = 17). METHOD: Whole body 3D analysis of both groups was conducted using a 14 camera VICON system. Spatio-temporal and kinematic variables were determined through a Plug-in-Gait model. SSW was obtained from an average of three trials equating to a total of ∼120 m walking. RESULTS: The group with Achondroplasia were 23 % slower (P < 0.001), had a 29 % shorter stride length (P < 0.001) and a 13 % higher stride frequency (P < 0.001) compared to controls. There were no differences in time normalised temporal measures of left toe off (P = 0.365), right heel contact (P = 0.442) or the duration of double support (P = 0.588) between groups. A number of discrete joint kinematic differences existed between groups, resulting in the group with Achondroplasia having more 'flexed' lower limbs than controls throughout the gait cycle. CONCLUSION: Differences in absolute spatio-temporal variables between groups is likely due to the shorter leg length of the group with Achondroplasia, while their more flexed position of the lower limbs may facilitate toe-clearance during the swing phase.

A quantitative description of self-selected walking in adults with Achondroplasia using the gait profile score.

BACKGROUND: Achondroplasia is characterised by a shorter appendicular limb-to-torso ratio, compared to age matched individuals of average stature (controls). Previous work shows gait kinematics of individuals with Achondroplasia differing to controls, but no global quantification of gait has been made in adults with Achondroplasia. AIM: The aim of this study was to quantify gait differences between a group of adult males with Achondroplasia and controls during self-selected walking (SSW) using the Gait Profile Score (GPS). DESIGN: Whole body motion analysis of 10 adults with Achondroplasia (22 ± 3 yrs) who had not undergone leg lengthening and 17 adult controls (22 ± 2 yrs) was undertaken using a 14 camera VICON system (100 Hz). For each group, fifteen root mean squared Gait Variable Scores (GVS, units °) were computed from lower limb kinematic data and then summed to calculate GPS (°). RESULTS: The group with Achondroplasia had higher GVSs than controls in 10 of the 15 measures (P < 0.05) with the largest differences found in ankle plantar/dorsiflexion (P < 0.001), knee flexion/extension (P < 0.001), and hip internal/external rotation (P < 0.001). The GPS value of the group with Achondroplasia was 64% higher than controls (11.4° (2.0) v 4.1° (1.8), P < 0.001). CONCLUSION: Gait is quantitatively different in adults with Achondroplasia compared to controls. The differences in GPS between groups are due to differences in joint kinematics, which are possibly manifested by maintaining toe-clearance during swing. Gait models derived from the anatomy of individuals with Achondroplasia may improve these data.

Bone health measured using quantitative ultrasonography in adult males with muscular dystrophy.

OBJECTIVES: To compare muscle and bone health markers in adult males (aged 20-59 yrs) with and without muscular dystrophy (MD). METHODS: Participants included 11 Fascioscapulohumeral (FSH), 11 Becker's (Be), 9 limb girdle (LG), 11 Duchenne (DMD), and 14 non-dystrophic controls (CTRL). Physical activity was assessed using Bone (BPAQ) and disability specific (PASIPD) questionnaires. Bone QUS provided T- and Z scores from the Distal Radius (DR) and Mid-shaft tibia (MST). Tibialis anterior cross sectional area (TAACSA) was measured using B-mode ultrasound. Grip strength was measured in all but DMD. RESULTS: Physical activity was lower in DMD, FSH and BeMD than CTRL (P<0.05), and lower in DMD than other MDs (P<0.01). T and Z scores were lower in DMD and Be than CTRL (DR, P<0.05); and lower in DMD than CTRL, LG, and FSH (MST, P<0.01). TAACSA and grip strength was 35-59% and 50-58% smaller in MD than CTRL, respectively (P<0.01). Within MD, BPAQ correlated with bone QUS measures (r=0.42-0.38, P<0.01). PASIPD correlated with grip strength (r=0.65, P<0.01) and TAACSA (r=0.46, P<0.01). CONCLUSION: Muscle size, strength, and bone health was lower in adult males with MD compared to adult males without MD, the extent of this is partially determined by physical activity.

Combined effects of body composition and ageing on joint torque, muscle activation and co-contraction in sedentary women.

This study aimed to establish the interplay between body mass, adiposity, ageing and determinants of skeletal muscle strength. One hundred and two untrained healthy women categorised by age into young (Y) (mean ± SD, 26.7 ± 9.4 years) vs. old (O) (65.1 ± 7.2 years) were assessed for body fat, lean mass, plantar flexion and dorsiflexion maximum voluntary isometric contraction (MVC) torque, muscle activation capacity and antagonist muscle co-contraction. MVC torque normalised to body mass in the obese group was 35 and 29 % lower (p < 0.05) in Y and 34 and 31 % lower (p < 0.05) in O, compared with underweight and normal weight individuals, respectively. Y with ≥40 % body fat had significantly lower activation than Y with <40 % body fat (88.3 vs. 94.4 %, p < 0.05), but O did not exhibit this effect. Co-contraction was affected by ageing (16.1 % in O vs. 13.8 % in Y, p < 0.05) but not body composition. There were significant associations between markers of body composition, age, strength and activation capacity, with the strongest correlation between muscle strength and total body mass (r (2) = 0.508 in Y, p < 0.001, vs. r (2) = 0.204 in O, p < 0.01). Furthermore, the age-related loss in plantar flexion (PF) MVC torque was exacerbated in obese compared to underweight, normal weight and overweight individuals (-0.96 vs. -0.54, -0.57 and -0.57 % per year, p < 0.05). The negative impact of adiposity on muscle performance is associated with not only muscular but also neural factors. Overall, the effects of ageing and obesity on this system are somewhat cumulative.

Gender differences in fascicular lengthening during eccentric contractions: the role of the patella tendon stiffness.

AIM: Elastic tendons have been suggested to attenuate fascicle lengthening during eccentric contractions; however, there is no in vivo evidence to support this hypothesis. Therefore, the aim of this study was to determine whether patella tendon stiffness modulates vastus lateralis (VL) fascicle lengthening during eccentric contractions in males and females. METHOD: Vastus lateralis and patella tendon properties were measured in males and females owing to previously reported intrinsic gender differences in tendon properties. During maximal voluntary eccentric knee extensions, VL fascicle lengthening and torque were recorded at every 10° (range of motion 20-90°). RESULTS: A significant correlation between maximal patella tendon stiffness and change in fascicle length (r=0.476, P=0.023) was observed. Similarly, there was a significant correlation between maximal Young's modulus and change in fascicle length (r=0.470, P=0.049). As expected, patella tendon stiffness and Young's modulus were significantly higher in males compared with females (P<0.05). Interestingly, change in VL fascicle length during the eccentric contractions was significantly greater in males compared with females (P<0.05). Based on patella tendon moment arm measurements, VL muscle-tendon unit elongation was estimated to be significantly greater in males compared with females (5.24 and 4.84 cm respectively). CONCLUSION: The significant difference in fascicle lengthening during eccentric contractions may be partly explained by the significantly higher patella tendon moment arm, patella tendon stiffness and Young's modulus found in males compared with females. The current study provides in vivo evidence to support the hypothesis that the tendon acts as a 'mechanical buffer' during eccentric contractions.