The impact of shoes versus ankle-restricted orthoses on sit-to-stand kinematics and centre of mass trajectories in adults with myelomeningocele.

BACKGROUND: Individuals with myelomeningocele (MMC) present with neurological and orthopaedic deficiencies, requiring orthoses during walking. Orthoses for counteracting dorsiflexion may restrict activities such as rising from a chair. RESEARCH QUESTION: How are sit-to-stand (STS) movements performed with ankle joint-restricted ankle-foot orthoses (AFO) and knee-ankle-foot orthoses with a free-articulated knee joint (KAFO-F)? METHODS: Twenty-eight adults with MMC, mean age 25.5 years (standard deviation: 3.5 years), were divided into an AnkleFree group (no orthosis or a foot orthosis) and an AnkleRestrict group (AFOs or KAFO-Fs). Study participants performed the five times STS test (5STS) while their movements were simultaneously captured with a three-dimensional motion system. Centre of mass (CoM) trajectories and joint kinematics were analysed using statistical parametric mapping. RESULTS: The AnkleRestrict group performed the STS slower than the AnkleFree group, median 8.8 s (min, max: 6.9, 14.61 s) vs 15.0 s (min, max: 7.5, 32.2 s) (p = 0.002), displayed reduced ankle dorsiflexion (mean difference: 6°, p = 0.044) (74-81 % of the STS cycle), reduced knee extension (mean difference: 14°, p = 0.002) (17-41 % of the STS cycle), larger anterior pelvic tilt angle (average difference: 11°, p = 0.024) (12-24 % of the STS cycle), and larger trunk flexion angle (on average 4°, p = 0.029) (6-15 % of the STS cycle). SIGNIFICANCE: The differences between the AnkleFree and AnkleRestrict groups in performing the STS seem consistent with the participants functional ambulation: community ambulation in the AnkleFree group, and household and nonfunctional ambulation with less hip muscle strength in the majority of the AnkleRestrict group. No differences in the 5STS CoM trajectories or the kinematics were found with respect to the AFO and KAFO-Fs groups. Because orthoses are constructed to enable walking, the environment needs to be adjusted for activities in daily living such as the STS movement.

Signs of perceptual disorder during movement were reliably assessed in children with cerebral palsy in Sweden.

AIM: The aim of this Swedish study was to evaluate the assessment of clinical signs of perceptual disorder in children with cerebral palsy (CP). METHODS: Three experienced raters assessed 56 videos of 19 children from 1 to 18 years of age with bilateral spastic CP, which were recorded by colleagues at an Italian hospital. Six signs were evaluated for inter-rater reliability and criterion validity. Clinical applicability was evaluated by assessing inter-rater reliability between 47 Swedish clinicians, who examined 15 of the videos during face-to-face and online education seminars. There were 41 physiotherapists, two occupational therapists and four doctors, with 1-37 years of clinical experience and a median of 10 years. RESULTS: The experienced raters demonstrated moderate to almost perfect inter-rater reliability (kappa 0.54-0.81) and criterion validity (0.54-0.87) for startle reaction, upper limbs in startle position, averted eye gaze and eye blinking. The clinicians recognised these signs with at least moderate reliability (0.56-0.88). Grimacing and posture freezing were less reliable (0.22-0.35) and valid (0.09-0.50). CONCLUSION: Four of the six signs of perceptual disorder were reliably recognised by experienced raters and by clinicians after education seminars. Extended education and larger study samples are needed to recognise all the signs.

Orthosis use and ambulation in adults with myelomeningocele after orthotic management from childhood.

BACKGROUND: Orthotic devices are required for walking in many individuals with myelomeningocele. Evidence concerning orthosis use is sparse, partly because of heterogeneity among groups and different definitions of the neurological level. OBJECTIVES: The objective of this study was to investigate ambulation regarding orthosis use and satisfaction with orthoses after intense orthotic management during childhood. STUDY DESIGN: The study design is a retrospective follow-up with a cross-sectional study at adult age. METHODS: Participants comprised 59 persons born in 1985 or later. Ambulation was categorized as community (Ca), household (Ha), nonfunctional (N-f), and nonambulation (N-a) groups. Orthosis use was registered at approximately 5 (Age5) and 12 (Age12) years of age and in adulthood (AdultAge). Satisfaction with orthoses was evaluated at AdultAge. RESULTS: At Age5, Age12, and AdultAge, orthoses were used by 100%, 98%, and 78% of participants, respectively. Ambulation deteriorated between Age5 and Age12 in 17% of participants and between Age12 and AdultAge in 46%. At AdultAge, 63% maintained their ambulatory function; and muscle function and hip and knee flexion contractures were strongly correlated with ambulation. The ambulation groups did not differ regarding satisfaction with device or services, except in 2 single items where the Ha group differed in ratings from the N-f and N-a groups. CONCLUSION: The high frequency of orthosis use and similar satisfaction in all ambulation groups emphasize that early planning and follow-up of orthosis treatment during growth are important for mobility in adulthood. Our results also underline the importance of a close assessment of each individual's condition.

Knee Flexion While Walking Exceeds Knee Flexion Contracture in Children with Spastic Cerebral Palsy.

Flexed knee gait is commonly related to contractures in children with cerebral palsy (CP). Therefore, knee position while walking was compared with passive knee extension and explored with respect to functional mobility. Gait was assessed with 3D motion analysis in 30 children with bilateral spastic CP, Gross Motor Function Classification System (GMFCS) levels I-III, and in 22 typically developing (TD) children. Knee angle at initial contact (KneeAngleIC) was greater than knee flexion in stance (MinKneeFlexSt) in all groups. MinKneeFlexSt exceeded knee contractures at GMFCS levels II and III. Both KneeAngleIC and MinKneeFlexSt were greater at GMFCS II and III than at GMFCS I and the TD group. The excessive knee flexion while walking at GMFCS II and III could not be explained by knee joint contractures. Functional mobility measured with the timed-up-and-go test took longer in children at GMFCS level III compared to the other groups, assumed to be explained by the energy-requiring flexed knee gait and spatial insecurity. Discriminating between passive knee extension at the physical assessment and maximum knee extension while weight bearing may contribute to further understanding of flexed knee gait and its causes in ambulating children with spastic bilateral CP.

Responses to Sensory Events in Daily Life in Children with Cerebral Palsy from a Parent Reported Perspective and in a Swedish Context.

The motor disorders of cerebral palsy (CP) are often accompanied by sensory disturbances, but knowledge of their relationship to motor functioning is sparse. This study explored responses to sensory events in relation to spastic subtype and motor functioning in children with CP. Parents of 60 children with CP (unilateral: 18, bilateral: 42) with GMFCS levels I:29, II:13, III:15 and IV:3 of mean age 12.3 years (3.7 SD) participated. The parents (n = 55) rated their children´s responses with the norm-referenced questionnaire Child Sensory Profile-2© (CSP-2©), Swedish version, incorporating nine sections and four sensory processing patterns/quadrants, and replied (n = 57) to two additional questions. On the CSP-2©, thirty (55%) of the children were reported to have responses "much more than others" (>2 SD) in one or more of the sections and/or quadrants and 22 (40%) in the section of Body Position, overrepresented by the children with bilateral CP. The additional questions revealed that a greater proportion of children at GMFCS levels III-IV compared to level I frequently were requested to sit/stand up straight (14/17 versus 6/26, p < 0.001) and were sound sensitive at a younger age (14/17 versus 10/26, p = 0.005). The findings of this study highlight the sensory aspects of motor functioning in children with spastic CP.

Physical function and activity, pain, and health status in adults with myelomeningocele after orthotic management from childhood: a descriptive study.

BACKGROUND: Individuals with myelomeningocele (MMC) exhibit neurological deficits below the lesion level involving both motor and sensory functions. Ambulation and functional outcomes in patients offered orthotic management since childhood were investigated. METHODS: Physical function, physical activity, pain, and health status were assessed in a descriptive study. RESULTS: Of 59 adults with MMC, aged 18-33 years, 12 were in the community ambulation (Ca), 19 in the household ambulation (Ha), six in the non-functional (N-f), and 22 in the non-ambulation (N-a) groups. Orthoses were used by 78% (n = 46), i.e., by 10/12 in the Ca, 17/19 in the Ha, 6/6 in the N-f, and 13/22 in the N-a groups. In the ten-metre walking test, the non-orthosis group (NO) walked faster than those wearing ankle-foot orthoses (AFOs) or free-articulated knee-ankle-foot orthoses (KAFO-Fs), the Ca group faster than the Ha and N-f groups, and the Ha group faster than the N-f group. In the six-minute walking test, the Ca group walked farther than the Ha group. In the five times sit-to-stand test, the AFO and KAFO-F groups required longer than the NO group, and the KAFO-F group longer than the foot orthosis (FO) group. Lower extremity function with orthoses was higher in the FO than the AFO and KAFO-F groups, higher in the KAFO-F than the AFO group, and higher in the AFO group than in those using trunk-hip-knee-ankle-foot orthoses. Functional independence increased with ambulatory function. Time spent in physical recreation was higher in the Ha than the Ca and N-a groups. There were no differences between the ambulation groups in rated pain or reported health status. CONCLUSION: The physical function results in persons with MMC improve our understanding of this population's heterogeneity and shed light on the importance of individualized orthotic management. The similarities between the various ambulatory levels in physical activity, pain, and health status may mirror opportunities to achieve equal results regardless of disability level. A clinical implication of the study is that orthotic management is likely to be beneficial for the patient with MMC of which the majority used their orthoses for most time of the day.

Evaluation of Knee Position Sense in Children with Motor Disabilities and Children with Typical Development: A Cross-Sectional Study.

BACKGROUND: In children with motor disabilities, knee position during walking is often of concern in rehabilitation. This study aimed to investigate knee joint position sense. Thirty-seven children with Cerebral Palsy (CP), 21 with Myelomeningocele (MMC), 19 with Arthrogryposis (AMC), and 42 TD children participated in the study. Knee joint position sense, i.e., the difference between the criterion angle and the reproduced angle (JPS-error), was assessed in sitting while 3D motion capture was recorded at flexed knee 70 (Knee70), 45 (Knee45), and 20 (Knee20) degrees, and after three seconds at maintained criterion angle (CAM) and maintained reproduced angle (RAM). No differences were found between the groups in JPS-error, CAM, and RAM. At Knee70, CAM differed between the right and left legs in the TD group (p = 0.014) and RAM in the MMC group (p = 0.021). In the CP group, CAM was greater than RAM at Knee70 in the left leg (p = 0.002), at Knee45 in both legs (p = 0.004, p = 0.025), and at Knee20 in the right leg (p = 0.038). Difficulties in maintaining the knee position at CAM in the CP group sheds light on the need for complementary judgments of limb proprioception in space to explore the potential influence on knee position during walking.

Working Memory in Navigational and Reaching Spaces in Typically Developing Children at Increasing School Stages.

BACKGROUND: Based on studies of children with motor disabilities on topographic working memory (TWM), no influence of age was reported. The only differences were in the degree of mobility and exploration of the environment. The more active a child was in exploring the environment, the less his/her TWM was poor. However, in typically developing children (TD), exploration of the environment increases with increasing age, and age-related effects have been described. Here, we aim at investigating TWM considering age in TD with the additional question of whether WM in the reaching space differed from that in the navigational space requiring body movements. We hypothesized that WM in both spaces would improve correspondingly with increasing age, assuming that the greater the autonomy in exploring the environment, the better TWM becomes. METHOD: 120 children (5-16 years old) performed the Corsi Block-Tapping test (CBT) and the Walking Corsi test (WalCT). RESULTS: Statistical analyses evidenced significantly increasing WalCT and CBT spans between each school stage, except in the CBT span between middle stage (MS) and upper stage (US). CBT spans were significantly higher than in the WalCT in the pre-school, lower stage, and MS, with the CBT span increasing until MS, which is sufficient for using spatial orientation strategies effectively. CONCLUSIONS: When navigation is gradually controlled, a child may be able to pay increasingly more attention to wayfinding and behavior in traffic. Since the US group even presented as good in the WalCT as young adults living in metropolitan environments, assuming that children may gain spatial orientation from having opportunities to move in their surroundings, this is also relevant for children with motor disabilities.

Locomotion and Topographical Working Memory in Children With Myelomeningocele and Arthrogryposis Multiplex Congenita.

Background: In children with myelomeningocele (MMC) and arthrogryposis multiplex congenital (AMC), adequate rehabilitation measures are accessible with the goal of attaining the utmost motor development. However, there is a lack of knowledge as to how children develop navigation utilizing their locomotion abilities. The aim of the present study was to explore topographic working memory in children with MMC and AMC. Methods: For this purpose, we assessed 41 children with MMC and AMC, assigned an ambulation group, and 120 typical developing (TD) children, with mean ages of 11.9, 10.6, and 9.9 years, respectively. All groups performed a topographic working memory test while moving in a walking space and a visuospatial working memory test in a reaching space. Children with MMC and AMC also performed a test to measure their ability to reason on visuospatial material, Raven's Coloured Progressive Matrices. Results: The topographic working memory span was shorter in the MMC group than in the TD group. In general, all ambulation groups had a shorter topographic working memory span than the TD group. The visuospatial working memory span was shorter in the non-ambulation group than in the TD group. Scores from the visuospatial reasoning test were lower in the non-ambulation group than in the community ambulation group. Conclusions: Even though a higher cognitive score was found in the community ambulation group than in the non-ambulation group, topographic working memory was affected similarly in both groups. Including children who develop community ambulation in therapy programs containing aspects of navigation may gain even children with low levels of MMC and AMC. These results evidenced the importance of motor development and navigational experience gained through direct exploration of the environment on topographic memory.

Motor function at increasing postural demands in children with bilateral cerebral palsy.

BACKGROUND: Among children with cerebral palsy (CP) some choose to be mobile by crawling or walking on their knees despite some bipedal walking ability. This motor behavior raises questions; so, we wanted to enhance understanding of the child's choice of mobility. AIM: To explore gross motor abilities in positions with various postural demands focusing on floor mobility among children with CP. DESIGN: A cross-sectional observational study. SETTING: The study was performed at the Neuropediatric Outpatient Department at Karolinska University Hospital. POPULATION: Thirty-six children with bilateral CP, median age 11.2 years, functioning at Gross Motor Function Classification System (GMFCS) level I-IV. METHODS: Motor skills using the Gross Motor Function Measure (GMFM-88), and lower leg muscles strength in hip flexors, knee extensors, ankle dorsiflexors and plantarflexors with a hand-held dynamometer were assessed. A Kruskal-Wallis Test with post-hoc Bonferroni corrections were used to compare GMFM percentage (%) scores and muscle strength between the GMFCS levels. RESULTS: GMFM-88 (%) scores in walking (E) and standing (D) dimensions were significantly higher at GMFCS levels I and II, compared to levels III and IV. In crawling and kneeling (C) level I achieved higher score than levels III and IV, and in sitting (B) than level IV. Muscle strength values in the plantarflexors were significantly higher at GMFCS level I compared to level II. CONCLUSIONS: As expected the children at GMFCS III performed less than those at GMFCS II at high postural demands in GMFM dimensions including standing and walking. Identical GMFM-scores in dimension C confirm similar motor function in items including kneeling and knee walking. Since lower limb muscle strength was similar, the difference in postural behavior between the groups at high level motor activities may be associated with sensorimotor disturbances along with the children's motor disorder. CLINICAL REHABILITATION IMPACT: The GMFM-88, in particular dimension C, including kneeling and walking items can be used as an identification of preference of floor mobility in children with CP. Awareness and understanding of how postural positions affect movement is of importance for prognosis, and physiotherapy.

Topographical Working Memory in Children with Cerebral Palsy.

Forty children with cerebral palsy (CP) and 120 typical developing children (TD) performed a topographic working memory (WalCT) test requiring to move their body in a walked vista-space and a visuo-spatial test (CBT) requiring just reaching movements. WalCT score was significantly higher in GMFCS II/III than in TD. CBT score was significantly lower in GMFCS I than in III/IV but lower than TD in all CP groups. Similar results in WalCT between GMFCS I and TD and GMFCS II and III/IV respectively indicate that mobility is associated with topographic working memory. Differently in CBT, the absence of bodily movement allows using different cognitive strategies. Children should be provided with opportunities and active participation to enhancing spatial awareness and navigational skills.

Head and Trunk Movements During Turning Gait in Children with Cerebral Palsy.

Thirty children with cerebral palsy (CP) and 22 typical developing (TD) were tested with 3D-gait analysis. At turning, trunk rotation was larger in CP2 (GMFCS II) than in TD and CP1 (GMFCS I), and head flexion was larger in CP3 (GMFCS III) than TD. Maximum head and trunk flexion values during the entire trial were larger in CP3 than in the other groups, and trunk flexion was larger in CP2 than in TD. Trial time increased with GMFCS-level. Less trunk rotation than TD and CP1 reflects spatial insecurity in CP2, which in CP3 is compensated by the walker. The flexed head and trunk in CP3 and trunk in CP2 may reflect deficits in proprioception and sensation requiring visual control of the lower limbs.

Health-related quality of life and orthosis use in a Swedish population with arthrogryposis.

BACKGROUND: Joint contractures are the main characteristics for children with arthrogryposis multiplex congenita. Orthoses are often used to enable or facilitate walking. OBJECTIVES: To describe health-related quality of life in children with arthrogryposis multiplex congenita and satisfaction with orthoses in those using orthoses. STUDY DESIGN: Cross-sectional study. METHODS: A total of 33 children with arthrogryposis multiplex congenita participated in the study. Questionnaires were used which measured health-related quality of life (Child Health Questionnaire-Parent Form and EQ-5D youth), mobility and self-care (Paediatric Evaluation of Disability Inventory) and satisfaction with orthoses (Quebec User Evaluation of Satisfaction with Assistive Technology 2.0). Children were divided into groups based on the use of orthoses: Ort-D were dependent on orthoses for walking, Ort-ND used orthoses but were not dependent on them for walking and Non-Ort did not use orthoses. RESULTS: Children with arthrogryposis multiplex congenita had significantly lower Child Health Questionnaire scores in 9 of 12 subscales compared to healthy controls. The children's reported perceived health with EQ-5D youth did not show any difference between children using orthoses or children using only shoes. Paediatric Evaluation of Disability Inventory showed less mobility in Ort-D than in Non-Ort. In total, both orthosis groups were 'quite satisfied' with their orthoses. CONCLUSION: Child Health Questionnaire-physical functioning was lowest in children who were dependent on orthoses (Ort-D) for walking. Both Ort-D and Ort-ND were similar satisfied with their orthoses. Clinical relevance This study contributes to knowledge about health-related quality of life in a group of ambulatory children with arthrogryposis multiplex congenita. For children using orthoses, it is relevant to capture their opinion about their orthoses but a questionnaire specifically for children should be developed.

The role of visual stimuli on standing posture in children with bilateral cerebral palsy.

BACKGROUND: In children with bilateral cerebral palsy (CP) maintaining a standing position can be difficult. The fundamental motor task of standing independently is achieved by an interaction between the visual, somatosensory, and vestibular systems. In CP, the motor disorders are commonly accompanied by sensory and perceptual disturbances. Our aims were to examine the influence of visual stimuli on standing posture in relation to standing ability. METHODS: Three dimensional motion analysis with surface electromyography was recorded to describe body position, body movement, and muscle activity during three standing tasks: in a self-selected position, while blindfolded, and during an attention-demanding task. Participants were twenty-seven typically-developing (TD) children and 36 children with bilateral CP, of which 17 required support for standing (CP-SwS) and 19 stood without support (CP-SwoS). RESULTS: All children with CP stood with a more flexed body position than the TD children, even more pronounced in the children in CP-SwS. While blindfolded, the CP-SwS group further flexed their hips and knees, and increased muscle activity in knee extensors. In contrast, the children in CP-SwoS maintained the same body position but increased calf muscle activity. During the attention-demanding task, the children in CP-SwoS stood with more still head and knee positions and with less muscle activity. CONCLUSIONS: Visual input was important for children with CP to maintain a standing position. Without visual input the children who required support dropped into a further crouched position. The somatosensory and vestibular systems alone could not provide enough information about the body position in space without visual cues as a reference frame. In the children who stood without support, an intensified visual stimulus enhanced the ability to maintain a quiet standing position. It may be that impairments in the sensory systems are major contributors to the difficulties to stand erect in children with CP.

Influence of External Visual Focus on Gait in Children With Bilateral Cerebral Palsy.

PURPOSE: To explore whether focusing a target influenced gait in children with cerebral palsy (CP) and typical development (TD). METHODS: Thirty children with bilateral CP (Gross Motor Function Classification System [GMFCS] I-III) and 22 with TD looked at a light at walkway end (Gaze Target) while walking and returned (No Target). RESULTS: During Gaze versus No Target, children with TD reduced temporal-spatial parameters and movements in the sagittal (SPM) and transverse planes. In comparison, during Gaze Target, children in CP1 (GMFCS I) had larger trunk SPM, children in CP2 (GMFCS II) larger neck (SPM), and children in CP3 (GMFCS III) greater head and neck frontal plane movements, and reduced cadence and single support. CONCLUSIONS: Focusing a target altered gait in children with CP. Children in CP1 reduced movements similar to children with TD, children in CP2 behaved nearly unchanged, whereas children in CP3 reduced movements and temporal-spatial parameters, potentially as a consequence of lack of sensory information from lower limbs.

The use of orthoses and gait analysis in children with AMC.

PURPOSE: Arthrogryposis multiplex congenita (AMC) can be described as a complex condition characterized by deformed joints with an intact sensory system. Consequences of muscle weakness and joint contractures in the lower limbs influence walking ability. With orthoses most children achieve functional ambulation. Based on four studies, the aim of this article was to describe gait pattern wearing habitual orthoses, to quantify quiet standing, to test and describe a new orthosis and compare gait differences with regular orthoses, in children with AMC. METHODS: In total 83 children, of which 35 with AMC took part in the studies. All children had underwent clinical examination. Based on joint range of motion and muscle strength they had been prescribed various orthosis types, ranging from insoles to knee-ankle-foot orthoses with locked knee joints. 3D gait and motion analysis was performed during standing and walking with 34 reflective markers aligned with anatomical landmarks. RESULTS: The findings are presented with respect to each of the included studies. CONCLUSIONS: According to the positive subjective impressions from parents and children, the clinical experiences of our research group, and the objective results from the gait assessments, continuously wearing of orthoses in persons with AMC is recommended.

Muscle strength does not explain standing ability in children with bilateral spastic cerebral palsy: a cross sectional descriptive study.

BACKGROUND: In bilateral cerebral palsy (CP) muscle strength is considered important for development of gross motor functions, but its influence on standing ability has not been explored. Our aims were to examine muscle strength with respect to the ability to stand with (SwS) or without (SwoS) hand support, asymmetrical weight bearing (WB), and whether the ability to produce strength was influenced by different seated conditions. METHODS: In this cross sectional descriptive study standing posture was recorded with 3D motion analysis, and muscle strength was measured with a hand-held dynamometer, in 25 children with bilateral CP, GMFCS levels II-III, SwS (n = 14, median age 11.4 years), or SwoS, (n = 11, median age 11.4 years). Strength measurements were taken in the hip flexors, knee extensors, dorsiflexors and plantarflexors, in two seated conditions; a chair with arm- and backrests, and a stool. RESULTS: Compared to SwoS, children SwS stood with a more flexed posture, but presented with equal strength in the hip flexors, dorsiflexors and plantarflexors, and with somewhat more strength in the knee extensors. Despite asymmetric WB during standing, both limbs were equally strong in the two groups. No differences in strength were measured between the two seated conditions. CONCLUSIONS: Despite challenges measuring muscle strength in CP, the lower limb muscle strength cannot be considered an explanatory factor for variations in standing in this group of children with bilateral CP. The findings rather strengthen our hypothesis that deficits in the sensory systems could be as determinant for standing as muscle weakness in children with bilateral spastic CP.