Figure 1 - uploaded by Yuichi Nishikawa
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The representative color map during isometric knee extension task. (upper panel) treadmill gait training, (lower panel) HAL gait training. Differences in the multi-channel surface electromyography spatial distribution patterns at each torque level were observed between the treadmill and HAL gait training in these representative data.
Source publication
Background: Despite the potential benefits, the effects of Hybrid Assistive Limb (HAL) gait training on changes in neuromuscular activation that accompany functional gains in individuals with spinal muscular atrophy (SMA) type III is not well known. In this article, we quantify the effects of HAL gait training on spatial muscle activity patterns in...
Contexts in source publication
Context 1
... a neuromuscular activation assessment, multi-channel SEMG was performed on the patient before and after both gait training. Figure 1 illustrates the representative multi-channel SEMG amplitude color maps for treadmill and HAL gait training. We normalized the CoV of force and RMS estimates to the values obtained pre-intervention for each torque level. ...
Context 2
... a neuromuscular activation assessment, multi-channel SEMG was performed on the patient before and after both gait training. Figure 1 illustrates the representative multi-channel SEMG amplitude color maps for treadmill and HAL gait training. We normalized the CoV of force and RMS estimates to the values obtained pre-intervention for each torque level. ...
Citations
... Only a few pieces of literature are available for rehabilitation management for patients with SMA [31], and more recent reports aim to study the training effect of wearable devices, namely, Hybrid Assistive Limb® (CYBERDYNE Inc. Ibaraki, Japan) [32,33]. Given that therapeutic intervention for SMA has entered a new era, rehabilitation program for SMA needs to be revisited, and the information of muscle involvement pattern may help us to develop a more e cient rehabilitation built upon logics. ...
BACKGROUND
Real-world data have shown variability in treatment responses to nusinersen in patients with 5q spinal muscular atrophy (SMA). It could be hypothesized that the degree of muscle impairment may confer to the drug response variability. Magnetic resonance imaging (MRI) is a useful tool to identify muscle involvement patterns in SMA; therefore, we investigated whether the magnitude of muscle involvement assessed by MRI at baseline can predict the change in gross motor function after the initiation of nusinersen treatment.RESULTSWe retrospectively assessed the clinical outcome of our treated cohort, in relevance to the thigh and pelvic MRI taken before the nusinersen treatment. A total of 16 patients with SMA types 2 and 3 (age = mean (SD); 9.2 (4.6) year) receiving nusinersen treatment were enrolled in the study. Hammersmith Functional Motor Scale-Expanded (HFMSE) was assessed to observe the change in motor function. The T1-weighted MR images of the pelvis and thigh were scored for muscle fatty infiltration and atrophy, and the interrater reliabilities of MR image evaluation were verified in all 16 images. The minimally clinically important difference (MCID) was considered as gaining at least 3 points of HFMSE from baseline. Of these 16 individuals, 14 had been treated for at least 15 months and had baseline data. At 15 months, seven individuals obtained MCID in HFMSE, and seven did not. Baseline muscle MRI score could not differentiate the two groups; however, individuals who obtained MCID had significantly less severe scoliosis. In addition, there was a significant and negative relationship between baseline MRI score and the change of score in HFMSE after 15 months of treatment. Further, baseline Cobb angle along with MRI score also correlated to the degree of change in motor function.CONCLUSION
It was indicated that the degree of muscle damage may confer the variability in response to nusinersen for individuals with SMA types 2 and 3. Muscle MRI score in addition to the severity of scoliosis assessed at baseline may help to predict the gross motor function change after the drug initiation.
Background
Spinal and bulbar muscular atrophy (SBMA) is a progressive neuromuscular degenerative disease characterized by the degeneration of lower motor neurons in the spinal cord and brainstem and neurogenic atrophy of the skeletal muscle. Although the short-term effectiveness of gait treatment using a wearable cyborg hybrid assistive limb (HAL) has been demonstrated for the rehabilitation of patients with SBMA, the long-term effects of this treatment are unclear. Thus, this study aimed to investigate the long-term effects of the continued gait treatment with HAL in a patient with SBMA.
Results
A 68-year-old man with SBMA had lower limb muscle weakness and atrophy, gait asymmetry, and decreased walking endurance. The patient performed nine courses of HAL gait treatment (as one course three times per week for 3 weeks, totaling nine times) for ~5 years. The patient performed HAL gait treatment to improve gait symmetry and endurance. A physical therapist adjusted HAL based on the gait analysis and physical function of the patient. Outcome measurements, such as 2-min walking distance (2MWD), 10-meter walking test (maximal walking speed, step length, cadence, and gait symmetry), muscle strength, Revised Amyotrophic Lateral Sclerosis Functional Assessment Scale (ALSFRS-R), and patient-reported outcomes, were evaluated immediately before and after gait treatment with HAL for each course. 2MWD improved from 94 m to 101.8 m, and the ALSFRS-R gait items remained unchanged (score 3) for approximately 5 years. The patient could maintain walking ability in terms of gait symmetry, walking endurance, and independence walking despite disease progression during HAL treatment.
Conclusion
The long-term gait treatment with HAL in a patient with SBMA may contribute to the maintenance and improvement of the gait endurance and ability to perform activities of daily living. The cybernics treatment using HAL may enable patients to relearn correct gait movements. The gait analysis and physical function assessment by a physical therapist might be important to maximize the benefits of HAL treatment.
Background:
Spinal muscular atrophy (SMA) is a hereditary disorder characterized by progressive muscle weakness and atrophy in children. However, less attention is paid to psychiatric symptoms of SMA parents. Attention to the psychiatric symptoms of parents of SMA children can improve the comprehensiveness of family support for SMA children, which is beneficial to the rehabilitation of SMA children. Here, we conducted a survey on the psychiatric symptoms of SMA parents and analyzed its relevant factors, with an attempt to inform the psychological support for SMA parents.
Methods:
The Symptom Checklist 90 (SCL-90) and a self-designed basic information (such as parent's gender, household area, place of residence, education background, etc.) questionnaire (in electronic questionnaire) were distributed to parents of SMA children aged 0-18 in a charity WeChat group sponsored by the Meier Advocacy & Support Center for SMA during the period from August 1 to August 31, 2021. Parents completed the electronic questionnaires by mobile phone or computer voluntarily. A total of 188 questionnaires were obtained, of which 182 were valid. Comparisons were performed with the SCL-90 adult norms as the standards. The potential correlations between the general data of SMA parents and children and abnormal factors in the SCL-90 for SMA parents were analyzed.
Results:
The SCL-90 factors somatization (1.56±0.80, P=0.002), depression (1.78±0.98, P<0.001), anxiety (1.58±0.87, P=0.007), fear (1.39±0.74, P=0.003), and sleep and eating problems (1.67±0.87, P=0.014) of SMA parents were significantly higher than the national norms. Place of residence was correlated with sleep and eating problems (r=0.158, P=0.033). Increasing age [odds ratio (OR) =1.012, P=0.014] and continuous home-living status (OR =0.360, P=0.031) of SMA children increased the risk of depression in their parents, and the lack of rehabilitation management in SMA children increased the risk of anxiety of their parents (OR =0.409, P=0.038). Non-urban residence (OR =2.602, P=0.017) and poor physical health (OR =0.163, P=0.031) were the relevant factors for the increased risk of sleep and eating problems in SMA parents.
Conclusions:
SMA parents have a higher risk of developing psychiatric symptoms problems compared with the general population. Increasing age and the continuous home-living status of SMA children increase the risk of depression in their parents, and the lack of rehabilitation management increase the risk of anxiety in SMA parents.
