Spinal muscular atrophy (SMA) is a neuromuscular disease that requires multidisciplinary medical care, including rehabilitation management. The emergence of a genetic therapy-based approach for SMA has markedly changed the disease course.Nonetheless, currently, updated physical therapy and rehabilitation are warranted for individuals with SMA in the era of gene therapy. In this review, we discuss the physical therapy and rehabilitation strategies currently performed for people with SMA, such as positioning and bracing, supported standing, management of musculoskeletal deformities, stretching, physical exercise training like aerobics and strengthening exercises, assistive devices, pulmonary rehabilitation, and dysphagia treatment.

The authors found that the fourth author's affiliation had been inadvertently omitted.

OBJECTIVE: To investigate the effects of mirror therapy using a tablet PC for post-stroke central facial paresis. METHODS: A prospective, randomized controlled study was performed. Twenty-one post-stroke patients were enrolled. All patients performed 15 minutes of orofacial exercise twice daily for 14 days. The mirror group (n=10) underwent mirror therapy using a tablet PC while exercising, whereas the control group (n=11) did not. All patients were evaluated using the Regional House–Brackmann Grading Scale (R-HBGS), and the length between the corner of the mouth and the ipsilateral earlobe during rest and smiling before and after therapy were measured bilaterally. We calculated facial movement by subtracting the smile length from resting length. Differences and ratios between bilateral sides of facial movement were evaluated as the final outcome measure. RESULTS: Baseline characteristics were similar for the two groups. There were no differences in the scores for the basal Modified Barthel Index, the Korean version of Mini-Mental State Examination, National Institutes of Health Stroke Scale, R-HBGS, and bilateral differences and ratios of facial movements. The R-HBGS as well as the bilateral differences and ratios of facial movement showed significant improvement after therapy in both groups. The degree of improvement of facial movement was significantly larger in the mirror group than in the control group. CONCLUSION: Mirror therapy using a tablet PC might be an effective tool for treating central facial paresis after stroke.
Facial Paralysis* ; Humans ; Mouth ; National Institutes of Health (U.S.) ; Outcome Assessment (Health Care) ; Prospective Studies ; Smiling ; Stroke*

OBJECTIVE: To compare the scores of the Bayley Scales of Infant Development second edition (BSID-II) and the third edition, Bayley-III, in children with suspected developmental delay and to determine the cutoff score for developmental delay in the Bayley-III. METHODS: Children younger than 42 months (n=62) with suspected developmental delay who visited our department between 2014 and 2015 were assessed with both the BSID-II and Bayley-III tests. RESULTS: The mean Bayley-III Cognitive Language Composite (CLC) score was 5.8 points higher than the mean BSID-II Mental Developmental Index (MDI) score, and the mean Bayley-III Motor Composite (MC) score was 7.9 points higher than the mean BSID-II Psychomotor Developmental Index (PDI) score. In receiver operating characteristic (ROC) analysis of a BSID-II MDI score < 70, Bayley-III CLC scores showed a cutoff of 78.0 (96.6% sensitivity and 93.9% specificity). In ROC analysis of a BSID-II PDI score < 70, the Bayley-III MC score showed a cutoff of 80. CONCLUSION: There was a strong correlation between the BSID-II and Bayley-III in children with suspected developmental delay. The Bayley-III identified fewer children with developmental delay. The recommended cutoff value for developmental delay increased from a BSID-II score of 70 to a Bayley-III CLC score of 78 and Bayley-III MC score of 80.
Child Development ; Child* ; Developmental Disabilities ; Humans ; ROC Curve ; Weights and Measures*

Objective: To develop and validate a scale to assess people’s attitudes and perspectives toward persons with disabilities. Methods: The three-stage development of the scale included a preliminary version drafted from the literature review and a nominal group process. Thereafter, the draft was examined further and revised through two rounds of Delphi survey by 16 disability experts. Lastly, the psychometric properties of the scale were assessed through an online survey of 1,359 employees at three university hospitals. Results: A 32-item scale, defined after two Delphi surveys, was refined into 14 items with four subcategories: community integration, discomfort, charitability, and sense of burdening. The Cronbach’s alpha coefficient was 0.839 and domain reliability from 0.638 to 0.845. Recent education on disabilities yielded more positive attitudes toward persons with disabilities. Meaningful acquaintances with disabilities yielded more positive attitudes toward persons with disabilities. Conclusion This newly developed scale that measures attitudes toward persons with disability is reliable and valid. A future use of the scale could be to measure attitudinal improvements toward persons with disabilities after awareness education.

Objective: To develop and validate a scale to assess people’s attitudes and perspectives toward persons with disabilities. Methods: The three-stage development of the scale included a preliminary version drafted from the literature review and a nominal group process. Thereafter, the draft was examined further and revised through two rounds of Delphi survey by 16 disability experts. Lastly, the psychometric properties of the scale were assessed through an online survey of 1,359 employees at three university hospitals. Results: A 32-item scale, defined after two Delphi surveys, was refined into 14 items with four subcategories: community integration, discomfort, charitability, and sense of burdening. The Cronbach’s alpha coefficient was 0.839 and domain reliability from 0.638 to 0.845. Recent education on disabilities yielded more positive attitudes toward persons with disabilities. Meaningful acquaintances with disabilities yielded more positive attitudes toward persons with disabilities. Conclusion This newly developed scale that measures attitudes toward persons with disability is reliable and valid. A future use of the scale could be to measure attitudinal improvements toward persons with disabilities after awareness education.

The population of adults diagnosed with cerebral palsy (CP) is increasing along with the survival rate of children born with the disability. Adults with CP need health services for the continued monitoring and management of their condition. Moreover, the development of additional health problems in adulthood increases the need for ongoing access to health services. Adults with CP manifest a higher rate of chronic health conditions and eventual decline in strength and functional reserve, deterioration in physical activity, increased risk of musculoskeletal complications, and gradual changes in swallowing ability. They are also reported to exhibit difficulty engaging socially and have a low health-related quality of life (QOL). However, there are a large number of adults with CP who cannot access medical services adequately and are therefore not effectively treated. To overcome these apparent challenges, we need to fully comprehend the healthcare needs of adults with CP to develop adult-focused health services. Further research is needed regarding the impact of physical activity, nutrition, sarcopenia, myeloradiculopathy, and swallowing function on QOL.
Adult ; Aging ; Cerebral Palsy ; Child ; Deglutition ; Delivery of Health Care ; Health Services ; Humans ; Motor Activity ; Quality of Life ; Sarcopenia ; Survival Rate

Objective: Aspiration is a major concern for children with dysphagia because aspiration has been known as the primary etiology leading to recurrent pneumonia, and severe impairment of lung function in pediatric populations.This study aimed to examine the differences in each temporal measure between children with and without aspiration and investigate the effect of bolus consistency on the temporal measures. Methods: Forty-two children ranging in age from 1 year to 3 years and 11 months were included in this study. A referral for a videofluoroscopic swallowing study (VFSS) was made. They were divided into two groups according to the presence or absence of aspiration. Sixteen children were aspirators, and twenty-six children were non-aspirators.Oropharyngeal temporal measures were analyzed using frame-by-frame analysis. Results: The delayed initiation of pharyngeal swallowing, delayed laryngeal closure, and poor bolus transition in the pharyngeal stage were associated with the risk of aspiration in children with dysphagia. The bolus consistency did not affect the oropharyngeal temporal measures. Conclusion The objective temporal data in this study, including the durations and onset of physiological events in relation to bolus movement, can be used for future research to compare the swallowing functions between groups of children with various etiologies and age ranges.

Objective: To investigate the reliability of knee extensor strength measurements using a supine hand-held dynamometer (HHD) anchoring frame in patients with limited physical activity. Although an HHD is suitable for bedside use, its inter-rater reliability is low because measurements can be influenced by tester strength. Methods: Maximal knee extensor isometric strength was measured using an HHD anchored to the supine frame. Three trials of three maximal contractions were assessed by two raters. Results: A total of 33 inpatients who were non-ambulatory due to acute illness participated in the study. The intraclass correlation coefficients were 0.974 (inter-rater) and 0.959 (intra-rater). The minimal detectable changes in intra- and inter-observer measurements were 29.46 N (24.10%) and 36.73 N (29.26%), respectively. The limits of agreement ranged from -19.79% to 24.81% for intra-rater agreement and from -21.45% to 37.07% for inter-rater agreement. Conclusion The portable dynamometer anchoring system can measure the isometric strength of the knee extensor reliably in the supine position, and could be used for measurements in patients who have difficulty visiting the laboratory and maintaining a seated posture.

Objective: Aspiration is a major concern for children with dysphagia because aspiration has been known as the primary etiology leading to recurrent pneumonia, and severe impairment of lung function in pediatric populations.This study aimed to examine the differences in each temporal measure between children with and without aspiration and investigate the effect of bolus consistency on the temporal measures. Methods: Forty-two children ranging in age from 1 year to 3 years and 11 months were included in this study. A referral for a videofluoroscopic swallowing study (VFSS) was made. They were divided into two groups according to the presence or absence of aspiration. Sixteen children were aspirators, and twenty-six children were non-aspirators.Oropharyngeal temporal measures were analyzed using frame-by-frame analysis. Results: The delayed initiation of pharyngeal swallowing, delayed laryngeal closure, and poor bolus transition in the pharyngeal stage were associated with the risk of aspiration in children with dysphagia. The bolus consistency did not affect the oropharyngeal temporal measures. Conclusion The objective temporal data in this study, including the durations and onset of physiological events in relation to bolus movement, can be used for future research to compare the swallowing functions between groups of children with various etiologies and age ranges.