Motor Function Measure Dimensions Research Articles

The impact of hamstring lengthening on stance knee flexion at skeletal maturity in ambulatory cerebral palsy.

This study reports the long-term outcomes of hamstring lengthening to treat flexed knee gait in children with ambulatory cerebral palsy (CP) after skeletal maturity. This retrospective longitudinal observational study used instrumented gait analysis (GA) <8 and >15 years old in children with bilateral CP. The primary variable was knee flexion in stance phase. Eighty children (160 limbs) were included; 49% were male, 51% female. Mean age at first GA was 6.0 (SD: 1.2) years and 19.6 (SD: 4.5) years at final GA. Mean follow-up was 13.7 (SD: 4.7) years. Children were classified as Gross Motor Function Classification System I-8, II-46 and III-26. Average Gross Motor Function Measure Dimension D was 72% (SD: 20%). Hamstring lengthenings occurred once in 82, twice in 54 and three times in 10 limbs. From initial to final GA, average knee flexion in stance was unchanged, 27.8° (SD: 14.8°) to final 27.0° (SD: 11.2°; P = 0.54). Knee flexion at foot contact was 39.6° (SD: 13.0°), improving to final GA of 30.7° (SD: 10.6°; P < 0.001). Initial gait deviation index was 65.8 (SD: 31.9), improving to final 78.9 (SD: 28.2; P < 0.001). Older age, males and concomitant plantar flexor lengthening predicted change toward more flexed knee gait. Hamstring lengthening did not lead to back-kneeing gait at maturity while maintaining childhood stance phase knee flexion. A subgroup still developed significant flexed knee gait posture and may have benefited from more aggressive treatment options. This outcome may also be impacted by diverse functional levels, etiologies and treatments of flexed knee gait.

Is Standing Function Improved After Orthopaedic Surgery in Children With Cerebral Palsy at GMFCS Levels III/IV?

Children with cerebral palsy (CP) at Gross Motor Function Classification System (GMFCS) levels III/IV are at risk for losses in standing function during adolescence and transition to adulthood. Multilevel surgery (MLS) is an effective treatment to improve gait, but its effects on standing function are not well documented. The objectives of our study were to describe standing function in children with CP classified as GMFCS levels III/IV and evaluate change after MLS. This retrospective study included children with CP (GMFCS III/IV) ages 6 to 20 years who underwent instrumented gait analysis. A subset who underwent MLS were evaluated for change. Primary outcome measures were Gross Motor Function Measure dimension D, gait velocity, functional mobility scale, and the Pediatric Outcomes Data Collection Instrument (PODCI). Additional impairment level measures included foot pressure, knee extension during stance phase of gait, and knee extension passive range of motion. Four hundred thirty-seven instrumented gait analysis sessions from 321 children with CP (ages 13.7±4.8y; GMFCS III-81%/IV-19%) were included. The GMFCS III group had higher Gross Motor Function Measure dimension D, gait velocity, PODCI scores, and better knee extension compared with the GMFCS IV group ( P <0.05); 94 MLS were evaluated for postoperative change 15.3±4.2 months after MLS. Children at GMFCS level III had improved PODCI scores ( P <0.05), better knee extension passive range of motion ( P <0.01), and improved coronal plane foot pressure ( P <0.05) post MLS. Maximum knee extension during stance and heel impulse improved significantly in both groups ( P <0.01). Standing function of children with CP at GMFCS IV was significantly more limited than at GMFCS III. After MLS, both groups (III/IV) showed improvement in impairment level outcomes (knee extension and foot position), whereas only those functioning at GMFCS III had improvement in activity/participation outcomes according to the PODCI. For children with CP at GMFCS levels IV, MLS may improve standing function, but appropriate goals related to assisted standing and measurement protocols sensitive to limited functional mobility should be adopted. Level III-retrospective comparative study.

Low-Intensity vs High-Intensity Home-Based Treadmill Training and Walking Attainment in Young Children With Spastic Diplegic Cerebral Palsy

ObjectiveTo compare the effect of low-intensity (LI) vs high-intensity (HI) treadmill training (TT) on walking attainment and overall walking activity in children with cerebral palsy (CP). DesignProspective, multisite, randomized controlled trial. SettingHomes of the participants. ParticipantsChildren with spastic diplegic CP, Gross Motor Function Classification System Level I and II, ages 14-32 months (N=19; male, n=8). InterventionsThe children were randomized to LI TT (2×/wk for 6wk) (n=10) and HI TT (10×/wk for 6wk) (n=9). The TT was carried out by the families with weekly instruction by the researchers. Main Outcome MeasuresChildren were assessed at study onset, post intervention, and 1 and 4 months post intervention with the Gross Motor Function Measure Dimension D/E (GMFM D/E), average strides per day and percentage of time spent walking with accelerometers, the Peabody Developmental Motor Scales-2 (PDMS-2), Pediatric Evaluation of Disability Index Mobility Scale, timed 10-m and 1-minute walk test, and Functional Mobility Scale. Blinding was conducted for GMFM D/E and PDMS-2. Linear mixed effects regression models were applied to all outcomes. ResultsNo significant between-group differences were found in any outcome measure at any of the time points. Children in the HI group did not show significant improvement immediately following the intervention in GMFM E (P=.061), while children in the LI group did (P=.003), but no statistically significant differences were detected over time (P=.71). Children in the HI group showed better walking independence on the Functional Mobility Scale at all postintervention assessments. ConclusionsA twice-weekly dosage was equally effective in improving skills related to walking compared with a 10×/wk program and can be more readily implemented into clinical practice.

Disease duration and disability in dysfeRlinopathy can be described by muscle imaging using heatmaps and random forests.

The manner in which imaging patterns change over the disease course and with increasing disability in dysferlinopathy is not fully understood. Fibroadipose infiltration of 61 muscles was scored based on whole-body MRI of 33 patients with dysferlinopathy and represented in a heatmap. We trained random forests to predict disease duration, Motor Function Measure dimension 1 (MFM-D1), and modified Rankin scale (MRS) score based on muscle scoring and selected the most important muscle for predictions. The heatmap delineated positive and negative fingerprints in dysferlinopathy. Disease duration was related to infiltration of infraspinatus, teres major-minor, and supraspinatus muscles. MFM-D1 decreased with higher infiltration of teres major-minor, triceps, and sartorius. MRS related to infiltration of vastus medialis, gracilis, infraspinatus, and sartorius. Dysferlinopathy shows a recognizable muscle MRI pattern. Fibroadipose infiltration in specific muscles of the thigh and the upper limb appears to be an important marker for disease progression. Muscle Nerve 59:436-444, 2019.

DUCHENNE MUSCULAR DYSTROPHY – IMAGING AND BIOMARKERS: P.37Upper extremity quantitative muscle ultrasound is related to disease severity in boys with Duchenne muscular dystrophy

With an increasing amount of non-ambulant boys with Duchenne muscular dystrophy (DMD), objective and child-friendly outcome measures are necessary to evaluate upper limb function in boys with DMD. The primary aim of this study was to assess the relation between upper extremity quantitative muscle ultrasound (QMUS) and disease severity in boys with DMD. Additionally, reference values of QMUS were collected in healthy boys and the effect of age on QMUS was compared between healthy boys and boys with DMD. Finally, reliability was assessed in a subgroup of both groups (12 healthy boys and 10 boys with DMD). Echo intensity (EI) and muscle thickness (MT) of the upper limb measured by QMUS were determined in 71 healthy boys and 43 boys with DMD (mean age 12.45 and 12.41 years). Clinical assessments (upper extremity muscle strength (MS), Brooke scale, Performance of upper limb (PUL) and Motor Function Measure Dimension 3 (MFM-D3)) were performed in boys with DMD only. The effect of age on QMUS was assessed using linear regression analysis. The relation between QMUS and clinical assessments was assessed using correlation coefficients. ICC's were constructed to determine inter-rater and test-retest reliability. EI was higher and increased with age in boys with DMD whereas in healthy boys EI was independent of age. MT increased with age in healthy boys whereas age did not influence MT in boys with DMD. A higher EI was related to decreased performance on Brooke scale, PUL and MS of shoulder abductors. A lower MT was related to decreased performance on Brooke scale and MS of shoulder abductors. This study found excellent inter-rater reliability of EI in healthy boys, which is in accordance with previous literature. Poor test-retest reliability of EI was found in both healthy boys and boys with DMD. QMUS of the upper extremity is related to disease severity in boys with DMD. Therefore, this study establishes QMUS of the upper extremity as an objective, relevant outcome measure in DMD which is not influenced by fatigue, verbal understanding or patient cooperation.

Progressive resistance training and mobility‐related function in young people with cerebral palsy: a randomized controlled trial

The aim of this study was to investigate whether individualized resistance training improves the physical mobility of young people with cerebral palsy (CP). Forty-eight participants with spastic diplegic CP (26 males, 22 females; mean age 18y 1mo, SD 1y 11mo) classified as level II or III on the Gross Motor Function Classification System were allocated randomly to progressive resistance training or usual-care control. Resistance training was completed twice weekly for 12 weeks at a community gymnasium under the supervision of a physiotherapist. Exercises were based on instrumented gait analysis and targeted muscles contributing to walking difficulties. Outcomes at 12 weeks and 24 weeks included objective measures of mobility (6-min walk test, instrumented gait analysis, and Gross Motor Function Measure dimensions D and E), participant-rated measures of mobility (Functional Mobility Scale and Functional Assessment Questionnaire), and muscle performance. The strength of targeted muscles increased by 27% (95% CI 8-46%) compared with control group. There were no between-group differences in any objective measure of mobility at 12 weeks (6-min walk test: mean difference 0.1m; 95% CI -21 to 21m) or at 24 weeks. Participant-rated mobility improved (Functional Mobility Scale at 5m: mean 0.6 units; 95% CI 0.1-1.1 units; Functional Assessment Questionnaire: 0.8 units; 95% CI 0.1-1.6 units) compared with control group at 12 weeks. Individualized progressive resistance training increased strength in adolescents and young adults with spastic diplegic CP. Despite participant-rated benefits, the increased strength did not result in objective improvements in mobility.

Responsiveness of the Motor Function Measure in Patients With Spinal Muscular Atrophy

ObjectiveTo assess the ability of the Motor Function Measure (MFM) to detect changes in the progression of spinal muscular atrophy (SMA). DesignObservational, retrospective, multicenter cohort study. SettingSeventeen departments of pediatric physical medicine. ParticipantsVolunteer patients with SMA (N=112) aged 5.7 to 59 years with no treatment other than physical therapy and nutritional or respiratory assistance. InterventionsNot applicable. Main Outcome MeasuresThe distributions of the MFM scores (total score and 3 subscores) were analyzed per SMA subtype. The relationships between scores and age were studied. The slopes of score changes (reflecting MFM responsiveness) were estimated in patients with at least 6 months' follow-up and 2 MFMs. Hypothetical sample sizes for specific effect sizes in clinical trial scenarios are given. ResultsIn 12 patients with SMA type 2 and 19 with SMA type 3 (mean ± SD follow-up, 25.8±19mo), there was a moderate inverse relationship between age and the MFM total score. Patients with less than 6 months' follow-up showed little score changes. Patients with longer follow-ups showed a slow deterioration (−0.9 points/y for type 2 and −0.6 points/y for type 3). Substantial responsiveness was obtained with the MFM Dimension 2 subscore (proximal and axial motricity) in patients with SMA type 2 (standardized response mean [SRM]=1.29), and with the MFM Dimension 1 subscore (standing and transfers) in patients with SMA type 3 aged 10 to 15 years (SRM=.94). ConclusionsIf further confirmed by larger studies, these preliminary results on the relative responsiveness of the MFM in SMA will foster its use in monitoring disease progression in patients who participate in clinical trials.

Measuring steady-state oxygen uptake during the 6-min walk test in adults with cerebral palsy

This study evaluated the feasibility of measuring steady-state oxygen uptake (VO2) during the 6-min walk test (6MWT) in adults with cerebral palsy (CP) who walk without support and whether there is construct validity for net 6MWT VO2 as a measure of their walking ability. Cardiorespiratory variables were assessed at rest and during the 6MWT in 15, independently ambulatory adults, 21-41 years old, with CP. The Gross Motor Function Measure dimensions D and E (GMFM-D and GMFM-E) quantified walking-related skills. Steady-state VO2 was achieved during the 6MWT. After controlling for body mass and speed, the net 6MWT VO2 was strongly related to GMFM-D (r=-0.58, ρ=0.03) and GMFM-E scores (r=-0.66, ρ=0.007). We conclude that for young adults with CP who walk without support, it is feasible to measure steady-state VO2 during the 6MWT and that the net 6MWT VO2 has construct validity as a measure of walking ability.

Motor assessment in patients with Duchenne muscular dystrophy

Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD) in a period of six months. Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC), and application of the Motor Function Measure (MFM). The patients were evaluated twice within a six-month interval. Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04). In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006). Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.

An Intensive Virtual Reality Program Improves Functional Balance and Mobility of Adolescents With Cerebral Palsy

To examine functional balance and mobility in adolescents with cerebral palsy classified at Gross Motor Function Classification System (GMFCS) level I following an intensive short-duration virtual reality (VR) intervention. Single-subject, multiple-baseline design with 4 adolescents. Outcomes included the Community Balance and Mobility Scale (CB&M), the 6-Minute Walk Test (6MWT), the Timed Up and Down Stairs, and the Gross Motor Function Measure Dimension E. Assessments were recorded 3 to 6 times at baseline, 5 times during intervention, and 4 times at follow-up. Daily 90-minute VR intervention was completed for 5 consecutive days. Visual, statistical, and clinical significance analyses were used. Statistically significant improvements were shown in all adolescents on CB&M and 6MWT. True change was recorded in all for the CB&M and in 3 for the 6MWT. Functional balance and mobility in adolescents with cerebral palsy classified at GMFCS level I improve with intense, short duration VR intervention, and changes are maintained at 1-month posttraining.

A Prospective Cohort Study of the Effects of Lower Extremity Orthopaedic Surgery on Outcome Measures in Ambulatory Children With Cerebral Palsy

Lower-extremity musculotendinous surgery is standard treatment for ambulatory children with deformities such as joint contractures and bony torsions resulting from cerebral palsy (CP). However, evidence of efficacy is limited to retrospective, uncontrolled studies with small sample sizes focusing on gait variables and clinical examination measures. The aim of this study was to prospectively examine whether lower-extremity musculotendinous surgery in ambulatory children with CP improves impairments and function measured by gait and clinical outcome tools beyond changes found in a concurrent matched control group. Seventy-five children with spastic CP (Gross Motor Function Classification System levels I to III, age 4 to 18 y) that underwent surgery to improve gait were individually matched on the basis of sex, Gross Motor Function Classification System level, and CP subtype to a nonsurgical cohort, minimizing differences in age and Gross Motor Function Measure Dimension E. At baseline and at least 12 months after baseline or surgery, participants completed gait analysis and Gross Motor Function Measure, and parents completed outcome questionnaires. Mean changes at follow-up were compared using analysis of covariance adjusted for baseline differences. Surgery ranged from single-level soft tissue release to multilevel bony and/or soft tissue procedures. At follow-up, after correcting for baseline differences, Gillette Gait Index, Pediatric Outcomes Data Collection Instrument Expectations, and Pediatric Quality of Life Inventory (PedsQL) Physical Functioning improved significantly for the surgical group compared with the nonsurgical group, which showed minimal change. On the basis of a matched concurrent data set, there was significant improvement in function after 1 year for a surgical group compared with a nonsurgical group as measured by the Gillette Gait Index, with few significant changes noted in outcome measures. Changes over 1 year are minimal in the nonsurgical group, supporting the possibility of ethically performing a randomized controlled trial using nonsurgical controls. Therapeutic level 2. Prospective comparative study.

A randomized clinical trial of strength training in young people with cerebral palsy

This randomized clinical trial evaluated the effects of a home‐based, six‐week strength‐training programme on lower limb strength and physical activity of 21 young people (11 females, 10 males; mean age 13 years 1 month, SD 3 years 1 month; range 8 to 18 years) with spastic diplegic cerebral palsy (CP) with independent ambulation, with or without gait aids; (Gross Motor Function Classification System levels I to III). Compared with the 10 controls, the 11 participants in the strength‐training programme increased their lower limb strength (combined ankle plantarflexor and knee extensor strength as measured by a hand‐held dynamometer) at 6 weeks (F(1,19)=4.58, p=0.046) and at a follow‐up 12 weeks later (F(1,18)=6.25, p=0.041). At 6 weeks, trends were also evident for improved scores in Gross Motor Function Measure dimensions D and E for standing, running and jumping, and faster stair climbing. A relatively short clinically feasible home‐based training programme can lead to lasting changes in the strength of key lower‐limb muscles that may impact on the daily function of young people with CP.