Background Assessment of pain is not routine, standardized, or well-understood in individuals with Duchenne muscular dystrophy (DMD), even though pain is a common problem reported by more than half of the patients with DMD. Previous studies in this area included multiple neuromuscular diseases with highly variable phenotypes. Therefore, our aim was to specifically focus on DMD and evaluate the comprehensive pain characteristics according to the disease stages, from ambulatory to late non-ambulatory. Methods This cross-sectional study was conducted in an out-patient pediatric rehabilitation clinic including 148 male participants with confirmed DMD (14.5 ± 5.3 years of age). Face-to-face interviews were conducted using a structured questionnaire concerning the pain frequency, duration, intensity, location, aggravating/relieving factors, pain interference (Brief Pain Inventory), pain phenotype (PainDETECT Questionnaire), and functional ability (DMD Functional Ability Self-Assessment Tool). Pain characteristics were analyzed according to the clinical stage: ambulatory (Amb), early non-ambulatory (ENA), and late non-ambulatory (LNA). Results Of the 148 participants who completed the assessment, 66 (44.6%) reported pain during the previous 4 weeks. There were no differences in the pain duration or intensity among the three groups. Pain location (Amb: calf, ENA: knee, LNA: lumbosacral region), aggravating factor (Amb: ambulation, ENA: transfer, LNA: sitting), and relieving factor (Amb: rest and massage, ENA and LNA: positional change) differed according to the clinical stage. Individuals in the LNA stage reported an increase in the frequency of pain and number of pain sites. The effect of pain on mood was also found to be greater in the LNA group than in the other clinical stages. Conclusion There is a change in the pain characteristics, including the location, aggravating/relieving factors, pain frequency, and pain interference, with the progress of the disease in patients with DMD. Thus, clinicians could more efficiently and critically assess and manage the patients’ pain based on these findings.

BACKGROUND Although exercise has benefits for motor function and quality of life in patients with parkinsonism, these patients have many barriers to exercise participation. Recently, the use of mobile apps has been highlighted as a remotely supervised exercise management strategy. OBJECTIVE This study aimed to evaluate the effects of home-based exercise management with a customized mobile app on exercise amount, physical activity, and quality of life in patients with parkinsonism. METHODS This was a prospective, open-label, single-arm, pilot study. Participants were encouraged to engage in an 8-week home-based exercise program delivered through a customized app. The primary outcome was the exercise amount. The secondary outcomes were International Physical Activity Questionnaire (IPAQ), Parkinson’s Disease Questionnaire-39 (PDQ-39), and Geriatric Depression Scale (GDS). RESULTS A total of 21 participants completed the intervention and assessment (mean age: 72 years; women: 17/21, 81%; men: 4/21, 19%). The participants reported a significant increase in the total amount of exercise (baseline: 343.33±206.70 min/week; 8-week follow up: 693.10±373.45 min/week; P<.001) and in the amount of each exercise component including stretching, strengthening, balance and cooperation, and oral-motor and vocal exercise after 8 weeks. The analysis of secondary outcomes revealed significant improvements in the IPAQ (P=.006), PDQ-39 (P=.02), and GDS (P=.04) scores. The usability of the program with the mobile app was verified based on the positive responses such as “intention to use” and “role expectation for rehabilitation.” CONCLUSIONS Exercise management with a customized mobile app may have benefits for improving exercise adherence, physical activity, depression, and quality of life in patients with parkinsonism. This supervised home-based, technology-based, reinforcing, and multimodal exercise management strategy should be recommend to patients with parkinsonism. In addition, this program may be useful as an alternative exercise management strategy during the COVID-19 pandemic. Additional clinical trials are needed to evaluate the efficacy of this exercise program in a large population and to confirm its disease-modifying effects.

Rationale:In recent years, the use of extracorporeal membrane oxygenation (ECMO) treatment for pediatric patients with cardiorespiratory failure has increased, with emphasis being given to the prevention of complications in ECMO-treated patients. Several studies have reported ECMO-related central nervous system complications, such as intracranial hemorrhage, cerebral infarction, and seizure. However, few cases of peripheral nerve injury have been reported in ECMO-treated adults; there have also been no reported cases of peripheral nerve injury in the pediatric population.Patient Concerns:Two pediatric patients aged 16 and 6 experienced motor weakness in the extremities after the insertion of ECMO equipment.Diagnoses:They were diagnosed with peripheral nerve injuries through an electrodiagnostic study that showed femoral/sciatic neuropathies and brachial plexopathy. Arteriography and doppler sonography was performed to find the cause of peripheral nerve injury, and this may be the results of vascular compromise and compressive injuries, respectively.Interventions:Surgical embolectomy was performed to remove thrombus in one patient. Two patients received orthosis, and physical therapy and occupational therapy were performed to prevent contracture and improve strength and functional use.Outcomes:Two pediatric patients showed a gradual improvement in motor power and function.Lessons:Through this case report, we present rare ECMO-related complications and emphasize the importance of early diagnosis and monitoring of peripheral nerve injury in ECMO-treated children.