Åsa Bartonek

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.

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.

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: 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: 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.

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.

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.

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.