Abstract Background Gait patterns in children with myelomeningocele (MMC) at various neurological levels have been described, both with and without orthotic support. Although the neurological level of the lesion serves as an important predictor of ambulatory potential, the expected walking ability is not always achieved, as additional factors such as spasticity may influence gait negatively. The aim of this study was to retrospectively compare gait patterns as assessed in childhood with those observed in adulthood. Methods Of 59 individuals with MMC aged 18 years or older, 29 had undergone three-dimensional gait analysis in childhood (Ch-GA). These data were retrospectively analysed and compared with findings from a subsequent adult gait analysis (Ad-GA). The mean (standard deviation) age at the time of Ch-GA was 11.6 (4.1) years and at Ad-GA 25.9 (3.9) years. The median (range) interval between assessments was 15.0 years (5.1–17.2). Results Twenty-two participants maintained independent, non-assisted walking (Group A), 5 had transitioned from independent walking to using a walking aid (Group B), and 2 used a walking aid at both Ch-GA and Ad-GA (Group C), with individualized orthotic prescriptions provided at both time points. In Group A, two of eleven kinematic variables and six of eleven kinetic variables in the hip, knee, and ankle showed deterioration, and walking speed had decreased. Functional ambulation declined from 18 community ambulators and 4 household ambulators (Ha) in childhood to 8 and 14, respectively, in adulthood. In Group B, analysed with only Gait Deviation Index (GDI), values were unchanged, but all temporospatial gait parameters had deteriorated. Functional ambulation decreased from five individuals classified as Ha to two Ha and three non-functional ambulators. The two individuals in Group C, who used a walker at both assessments, largely maintained the same GDI values and temporospatial parameters as in childhood. Conclusion Largely consistent with our original expectations, the findings indicate that gait patterns remain relatively stable from childhood to adulthood in individuals with MMC when supported by appropriate rehabilitation interventions, though some deterioration of gait and ambulation occurred. The results reflect gait-related changes that can be expected during growth in this population.

Abstract Background Individuals with arthrogryposis multiplex congenita (AMC) exhibit a range of modes of ambulation, from walking independently to requiring a wheelchair. Presence of joint contractures and muscle strength plays a crucial role, and, in some patients, orthoses are necessary to facilitate or enable walking. Methods Gait was assessed with a three-dimensional (3D) gait analysis, calculated as a gait deviation index (GDI) of nine kinematic variables, and compared between childhood and adulthood. Results A total of 12 persons, 8 with community and 4 with household ambulation, who had undergone a 3D gait analysis in childhood (CH) and as an adult (follow-up, FU) at the same gait laboratory were enrolled in the study. At the FU, three, five, and four participants respectively were categorized based on need of joint stabilization while walking as AMC1 using knee-ankle-orthoses (KAFOs) with locked knee joints, AMC2 using KAFOs with free-articulating knee joint or ankle–foot-orthoses (AFOs) and AMC3 using insoles or shoes. Two participants in AMC2 had changed from AFOs to insoles or shoes between CH and FU. There were no differences in joint contractures between the AMC groups at CH or FU. Two participants had orthopaedic surgery between CH and FU. The GDI of the leg with the lowest GDI score at CH vs FU was median [min, max] 55.67 [41.79, 65.14] vs 48.4 [42.67, 56.30] (p = 1.000) in AMC1, 81.25 [59.42, 84.12] vs 68.96 [47.68, 70.33] (p = 0.043) in AMC2, and 73.15 [43.94, 91.72] vs 73.46 [50.82, 75.24] (p = 1.000) in AMC3. Time and distance parameters of cadence, walking speed, step length, and step width did not differ between the CH and FU, nor were there differences in satisfaction with the device or the service at the FU. Conclusion A difference in the GDI was found in one of the AMC groups between childhood and adulthood that could not be explained by factors such as contractures or muscle strength. This study reflects that gait is maintained in ambulating persons with AMC who were offered an orthosis program that has been available from childhood into adulthood.

The aim of this Swedish study was to evaluate the assessment of clinical signs of perceptual disorder in children with cerebral palsy (CP). 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. 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). 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.

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.

BACKGROUND/OBJECTIVES:Proprioception and sensory disorders have been reported in children with arthrogryposis multiplex congenita (AMC) and myelomeningocele (MMC), but valid and reliable assessment tools are limited in accurately identifying the sensory aspects of motor disorders. This study aimed to investigate the somatosensory status in the feet and legs. An additional purpose of this study was to explore pain, skin irritations, and health status. METHODS:Nineteen children with AMC, twenty-three with MMC, and twenty-two typically developing (TD) children (7-18 years old) were tested using a somatosensory test battery in ankle kinesthesia and in identifying four different types of floors. RESULTS:In the AMC and MMC groups, the threshold to perceive the somatosensory stimuli was not achieved by all participants. MMC participants perceived somatosensory stimuli less than TD participants in all tests, with a higher level of the lesion and more affected ambulation. The MMC group identified one floor significantly less often than the TD group. The AMC group performed better than the MMC group in two-point discrimination, vibration sensation, and some light-touch pressure tests. There were no differences among the TD, AMC, and MMC groups in ankle kinesthesia. Pain was reported by four (21%) subjects in the AMC group and five (22%) in the MMC group, and skin irritations were reported by three (13%) participants in the MMC group. There was no difference among the TD, AMC, and MMC groups in health status as reported using the EQ-5D-Y visual analog scale. CONCLUSIONS:Although differences in sensory aspects were the most evident between the groups, assessments of activity and participation levels in the rehabilitation of children with disabilities are also recommended.

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.

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.

Persons with myelomeningocele (MMC) present with neurological and orthopaedic deficiencies, often requiring orthoses to stabilise joints during walking [1]. Rising from a chair, a functional task demanding high coordination, is a common daily living activity. However orthoses for counteracting dorsiflexion during walking, commonly used by persons with MMC, may interfere with the need for sufficient ankle dorsiflexion to rise from sitting to standing. How are sit-to-stand movements performed with ankle joint-restricted orthoses? Twenty-eight adults with MMC, mean age 25.5 (SD 3.4) years, were included. The group was divided based on orthosis type; No orthosis and Foot orthosis (NO/FO); ankle joint-restricted Ankle-Foot-Orthosis and ankle joint-restricted and free-articulated Knee-Ankle-Foot-Orthosis (AFO/KAFO-F). Lower limb muscle strength was evaluated using a manual scale from 0–5 [2]. Study participants performed the Five times sit-to-stand test (5STS) while 3D motion capture was recorded. Centre of mass (COM) trajectories and joint kinematics were extracted and analysed using statistical parametric mapping comparing groups [3]. With no difference in time to perform the 5STS and comparable knee extensor strength, the NO/FO group displayed less forward displacement of the COM than the AFO/KAFO-F group at 17-34% of the STS-cycle (p<0.001) (Fig. 1). On average, across all STS-repetitions, the AFO/KAFO-F group performed the stand-up part with an increased anterior pelvic tilt at 13-24% (p=0.024), started to extend the knees earlier at 18-42% and stood with less extended knees at the end of the rising phase (p=0.002) compared to the NO/FO group (Fig. 1). No differences were observed in ankle kinematics between groups. Fig. 1. The three upper figures show the mean ± SD for averaged sit-to-stand cycles of A) forward displacement of Centre of Mass, B) sagittal plane knee joint range of motion (RoM), and C) sagittal plane pelvis RoM during the Five times sit-to-stand test. The three lower figures (D, E, F) display the SPM-ANOVA plot for each of the above corresponding sit-to-stand cycles. The black line indicates the within-subject model; the thin red line indicates the critical threshold (F*).Download : Download high-res image (153KB)Download : Download full-size image The greater forward COM displacement and the increased anterior pelvic tilt during the rising phase in AFO/KAFO-F compared to NO/FO may be seen as compensatory strategies to compensate for weak hip extensors, combined with the restricted dorsiflexion ankle movement. The smaller knee extension observed in standing in the AFO/KAFO-F group compared to the NO/FO group may be associated with knee and hip contractures. However, this slight knee flexion may also be considered a contributing factor for enabling sagittal alignment of the trunk, by counteracting hip flexion related to hip extensor weakness.

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.

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. Physical function, physical activity, pain, and health status were assessed in a descriptive study. 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

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.

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.

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.

EDITORIAL article Front. Psychiatry, 24 March 2022Sec. Child and Adolescent Psychiatry https://doi.org/10.3389/fpsyt.2022.875868

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.

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.