Kinematic gait deficits at the trunk and pelvis: characteristic features in children with hereditary spastic paraplegia.

AIM: To examine the kinematic gait deviations at the trunk and pelvis of children with hereditary spastic paraplegia (HSP). METHOD: This exploratory observational study quantified gait kinematics for the trunk and pelvis from 11 children with HSP (7 males, 4 females) using the Gait Profile Score and Gait Variable Scores (GVS), and compared the kinematics to data from children with typical development using a Mann-Whitney U test. RESULTS: Children with HSP (median age 11y 4mo, interquartile range 4y) demonstrated large deviations in the GVS for the trunk and pelvis in the sagittal and coronal planes when compared to the gait patterns of children with typical development (p=0.010-0.020). Specific deviations included increased range of movement for the trunk in the coronal plane and increased excursion of the trunk and pelvis in the sagittal plane. In the transverse plane, children with HSP demonstrated later peaks in posterior pelvic rotation. INTERPRETATION: The kinematic gait deviations identified in this study raise questions about the contribution of muscle weakness in HSP. Further research is warranted to determine contributing factors for gait dysfunction in HSP, especially the relative influence of spasticity and weakness.

Children with cerebral palsy and periventricular white matter injury: does gestational age affect functional outcome?

This study aimed to determine differences in functional profiles and movement disorder patterns in children aged 4-12 years with cerebral palsy (CP) and periventricular white matter injury (PWMI) born >34 weeks gestation compared with those born earlier. Eligible children born between 1999 and 2006 were recruited through the Victorian CP register. Functional profiles were determined using the Gross Motor Function Classification System (GMFCS), Manual Abilities Classification System (MACS), Communication Function Classification System (CFCS), Functional Mobility Scale (FMS) and Bimanual Fine Motor Function (BFMF). Movement disorder and topography were classified using the Surveillance of Cerebral Palsy in Europe (SCPE) classification. 49 children born >34 weeks (65% males, mean age 8 y 9 mo [standard deviation (SD) 2 y 2 mo]) and 60 children born ≤ 34 weeks (62% males, mean age 8 y 2 mo [SD 2 y 2 mo]) were recruited. There was evidence of differences between the groups for the GMFCS (p=0.003), FMS 5, 50 and 500 (p=0.003, 0.002 and 0.012), MACS (p=0.04) and CFCS (p=0.035), with a greater number of children born ≤ 34 weeks more severely impaired compared with children born later. Children with CP and PWMI born >34 weeks gestation had milder limitations in gross motor function, mobility, manual ability and communication compared with those born earlier.

Psychometric properties of functional mobility tools in hereditary spastic paraplegia and other childhood neurological conditions.

AIM: To evaluate studies on the psychometric properties of measurement tools used to quantify functional mobility in children with hereditary spastic paraplegia (HSP) and other childhood neurological conditions. METHOD: Two independent reviewers identified measures previously used by clinicians to quantify functional mobility. Because our primary interest was HSP, the first search identified measurement tools in studies that included those with HSP. To enhance the generalizability, the second search examined the reliability, validity, and responsiveness of tools in children with a range of neurological conditions such as cerebral palsy, spinal muscular atrophy, Down syndrome, and traumatic brain injury. The Consensus-based Standards for the Selection of Health Measurement Instruments was used to rate the methodological quality of identified articles. RESULTS: The Gillette Functional Assessment Questionnaire (FAQ), the Functional Mobility Scale (FMS), the Gross Motor Function Measure (GMFM), the Rivermead Motor Assessment, and the Walking Index for Spinal Cord Injury II were identified for quantifying functional mobility. The FMS and GMFM were reliable, valid, and responsive to changes across a range of childhood neurological conditions. The FAQ was reliable and valid for measuring functional mobility in similar populations. INTERPRETATION: The FAQ, FMS, and GMFM are valid, reliable, and responsive measures in children with a range of neurological conditions.

The Functional Mobility Scale (FMS).

We devised a new Functional Mobility Scale (FMS) to describe functional mobility in children with cerebral palsy, as an aid to communication between orthopaedic surgeons and health professionals. The unique feature of the FMS is the freedom to score functional mobility over three distinct distances, chosen to represent mobility in the home, at school and in the wider community. We examined the construct, content, and concurrent validity of the FMS in a cohort of 310 children with cerebral palsy by comparing the FMS to existing scales and to instrumented measures of physical function. We demonstrated the scale to be both valid and reliable in a consecutive population sample of 310 children with cerebral palsy seen in our tertiary referral center. The FMS was useful for discriminating between large groups of children with varying levels of disabilities and functional mobility and sensitive to detect change after operative intervention.