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Abstract Background High-resolution manometry (HRM) can quantify swallowing pathophysiology to evaluate the status of the pharynx. Sequential 4-channel neuromuscular electrical stimulation (NMES) was recently developed based on the normal contractile sequences of swallowing-related muscles. This study aimed to examine the effects of sequential 4-channel NMES for compensatory application during swallowing and to observe the residual effects after the application of NMES using HRM. Results Sequential 4-channel NMES significantly improved the HRM parameters, with respect to the maximal pressure and area of the velopharynx (VP), maximal pressure and area of the mesopharynx (MP), and upper esophageal sphincter (UES) activation and nadir duration. Furthermore, the improvement in the pressure and area variables of the VP and MP showed a tendency to maintain even when measured after NMES, but there are no significant differences. Conclusions The present study suggests that the sequential 4-channel NMES application of the suprahyoid and infrahyoid muscles during swallowing improves the pressure, area, and time variables of the oropharynx, as measured by HRM, and it is likely that the effects may persist even after stimulation. Trial Registration Clinicaltrials.gov, registration number: NCT02718963 (initial release: 03/20/2016, actual study completion date: 06/24/2016, last release: 10/20/2020).

Abstract This prospective pilot study investigated the influence of body position on peak cough flow (PCF) during mechanical insufflation–exsufflation (MI-E) treatment in people with tetraplegia. Fifteen participants with cervical spinal cord injury (C-SCI) were randomized into two groups, which differed in the starting position, that is, the patients were either supine or reclined. Four sessions of MI-E in alternating positions with each session comprising three different maneuvers: five voluntary coughs, five MI-E-assisted coughs, and five MI-E-assisted with manual thrusts were performed with continuous airflow measurement reporting PCF from every cough. PCF was associated with the application maneuvers, total insufflation volume (TIV), and interaction between position and maneuvers but not with the application position. The estimated mean PCF was 1.808, 3.529, and 3.925 L/s when supine and 1.672, 3.598, and 3.909 L/s when reclined from voluntary cough, MI-E, and MI-E with manual thrust, respectively. The estimated PCF change compared to voluntary cough was 1.721 (95% CI, 1.603–1.838) L/s from the combined MI-E and 2.116 (95% CI, 2.005–2.228) L/s from the MI-E with manual thrust, calculated from the linear mixed-model analysis. PCF moderately correlated with TIV (R2 = 0.64). Therefore, either position can be used for C-SCI patients as long as MI-E can be performed with manual thrust and sufficient TIV is provided.

Abstract Background Dual-task training in Parkinson’s disease (PD) improves spatiotemporal gait parameters, cognition, and quality of life. Virtual reality (VR) has been used as a therapeutic tool for patients to participate in activities in a safe environment, engage in multisensory experiences, and improve motivation and interest in rehabilitation. This study aimed to investigate the feasibility of fully immersive VR exergames with dual-task components in patients with PD. Methods We developed VR exergames (go/no-go punch game, go/no-go stepping game, and number punch game) to improve habitual behavior control using motor–cognitive dual-task performance in patients with PD. The participants underwent 10 sessions 2–3 times a week, consisting of 30 min per session. The Unified Parkinson’s Disease Rating Scale, Timed Up and Go test (TUG) under single- and dual-task (cognitive and physical) conditions, Berg balance scale (BBS), Stroop test, trail-making test, and digit span were evaluated before and after intervention. The Simulator Sickness Questionnaire (SSQ) was used to assess VR cybersickness. Usability was assessed using a self-reported questionnaire. Results Twelve patients were enrolled and completed the entire training session. The mean age of participants was 73.83 ± 6.09 years; mean disease duration was 128.83 ± 76.96 months. The Hoehn and Yahr stages were 2.5 in seven patients and 3 in five patients. A significant improvement was observed in BBS and Stroop color–word test (p = 0.047 and p = 0.003, respectively). TUG time and dual-task interferences showed positive changes, but these changes were not statistically significant. The median SSQ total score was 28.05 (IQR: 29.92), 13.09 (IQR: 11.22), and 35.53 (IQR: 52.36) before, after the first session, and after the final session, respectively; the differences were not significant. Overall satisfaction with the intervention was 6.0 (IQR: 1.25) on a 7-point Likert-type scale. Conclusions Fully immersive VR exergames combined with physical and cognitive tasks may be used for rehabilitation of patients with PD without causing serious adverse effects. Furthermore, the exergames using dual-task components improved executive function and balance. Further development of VR training content may be needed to improve motor and dual-task performances. Trial registration NCT04787549 ( https://clinicaltrials.gov/ct2/show/NCT04787549 )

BackgroundIntensive care unit (ICU)-acquired weakness (ICU-AW) is one of the most common complications of post-ICU syndrome. It is the leading cause of gait disturbance, decreased activities of daily living, and poor health-related quality of life. The early rehabilitation of critically ill patients can reduce the ICU-AW. We designed a protocol to investigate the feasibility and safety of conventional rehabilitation with additional in-bed cycling/stepping in critically ill patients.MethodsThe study is designed as a single-center, single-blind, pilot, randomized, parallel-group study. After the screening, participants are randomly allocated to two groups, stratified by mechanical ventilation status. The intervention group will be provided with exercises of in-bed cycling/stepping according to the level of consciousness, motor power, and function in addition to conventional rehabilitation. In contrast, the control group will be provided with only conventional rehabilitation. The length of intervention is from ICU admission to discharge, and interventions will be conducted for 20 minutes, a maximum of three sessions per day.ResultsThe outcomes are the number and percentage of completed in-bed cycling/stepping sessions, the duration and percentage of in-bed cycling/stepping sessions, and the number of cessations of in-bed cycling/stepping sessions, the interval from ICU admission to the first session of in-bed cycling/stepping, the number and percentage of completed conventional rehabilitation sessions, the duration and percentage of conventional rehabilitation sessions, the number of cessations of conventional rehabilitation sessions, the number of adverse events, level of consciousness, functional mobility, muscle strength, activities of daily living, and quality of life.DiscussionThis study is a pilot clinical trial to investigate the feasibility and safety of conventional rehabilitation with additional in-bed cycling/stepping in critically ill patients. If the expected results are achieved in this study, the methods of ICU rehabilitation will be enriched.Trial registrationclinicialtrials.gov, Clinical Trials Registration #NCT05868070.

The survival rate of children admitted in the neonatal intensive care unit (NICU) after birth is on the increase; hence, proper evaluation and care of their neurodevelopment has become an important issue. Neurodevelopmental assessments of individual domains regarding motor, language, cognition, and sensory perception are crucial in planning prompt interventions for neonates requiring immediate support and rehabilitation treatment. These assessments are essential for identifying areas of weakness and designing targeted interventions to improve future functional outcomes and the quality of lives for both the infants and their families. However, initial stratification of risk to select those who are in danger of neurodevelopmental disorders is also important in terms of cost-effectiveness. Efficient and robust functional evaluations to recognize early signs of developmental disorders will help NICU graduates receive interventions and enhance functional capabilities if needed. Several age-dependent, domain-specific neurodevelopmental assessment tools are available; therefore, this review summarizes the characteristics of these tools and aims to develop multidimensional, standardized, and regular follow-up plans for NICU graduates in Korea.

Objective To systematically translate the Duchenne muscular dystrophy Functional Ability Self-Assessment Tool (DMDSAT) into Korean and verify the reliability and validity of the Korean version (K-DMDSAT). Methods The original DMDSAT was translated into Korean by two translators and two pediatric physiatrists. A total of 88 patients with genetically confirmed Duchenne muscular dystrophy (DMD) participated in the study. They were evaluated using the K-DMDSAT once as a self-assessment and once by an interviewer. The interviewer evaluated the K-DMDSAT again 1 week later using a test-retest approach. The intraclass correlation coefficient (ICC) was used to verify the interrater and test-retest reliabilities. Pearson correlation analysis between the K-DMDSAT and the Brooke or Vignos scales were used to assess validity. Results The total score and all domains of the K-DMDSAT showed excellent interrater and test-retest reliability, with an ICC for total scores of 0.985 and 0.987, respectively. All domains had an ICC >0.90. From the Pearson correlation analysis, the total K-DMDSAT score was significantly correlated with the Vignos and Brooke scales (r=0.918 and 0.825, respectively; p

Objective To evaluate the current status of pain severity and quality of life (QoL) in patients with complex regional pain syndrome (CRPS), and to assess both their perceived needs and any unmet needs of current rehabilitation services. Methods A single-center questionnaire-based survey was conducted on 47 patients with CRPS who were diagnosed based on Budapest’s criteria. It collected demographic and clinical data, and the structured questionnaire included the Brief Pain Inventory (BPI), the Korean version of the World Health Organization Disability Assessment Schedule II (WHODAS-K II), as well as the 5-Level EuroQol-5D (EQ-5D-5L) for measuring the QoL. Results The average value of BPI and WHODAS-K II were 7.69%±2.26% and 70.49%±19.22%, respectively. In the evaluation of their perceived needs and unmet needs for rehabilitation, patients had the highest rehabilitation needs in terms of pain (95.74%), followed by bodyaches (80.85%). Regarding their unmet needs, patients had the highest unmet needs in terms of memory impairment (83.33%), followed by weight management (72.00%). According to the regression analysis, only the overall BPI was significantly associated with QoL (p=0.01), and a higher BPI value led to poorer results for QoL. Conclusion In Korea, patients with CRPS do not receive adequate rehabilitation, and they are not satisfied with current received treatments. A more structured and individualized rehabilitation treatment plan is required to manage every aspect related to chronic pain, and provision should be made for improved care guidelines for future CRPS management.

Vanishing white matter (VWM) disease is an autosomal recessive disorder that affects the central nervous system of a patient, and is caused by the development of pathogenic mutations in any of the EIF2B1-5 genes. Any dysfunction of the EIF2B1-5 gene encoded eIF2B causes stress-provoked episodic rapid neurological deterioration in the patient, followed by a chronic progressive disease course. We present the case of a patient with an infantile-onset VWM with the pre-described specific clinical course, subsequent neurological aggravation induced by each viral infection, and the noted consequent progression into a comatose state. Although the initial brain magnetic resonance imaging did not reveal specific pathognomonic signs of VWM to distinguish it from other types of demyelinating leukodystrophy, the next-generation sequencing studies identified heterozygous missense variants in EIF2B3, including a novel variant in exon 7 (C706G), as well as a 0.008% frequency reported variant in exon 2 (T89C). Hence, the characteristic of unbiased genomic sequencing can clinically affect patient care and decisionmaking, especially in terms of the consideration of genetic disorders such as leukoencephalopathy in pediatric patients.

BACKGROUND: Mechanical insufflation-exsufflation (MI-E) and manually assisted cough are frequently employed cough augmentation methods for enhancing cough efficiency in individuals with cervical spinal cord injury (CSCI). This study aimed to evaluate the synergistic impact of combining manually assisted cough and MI-E on cough peak flow in subjects with CSCI and identify their related factors. METHODS: Fifteen subjects with CSCI with cough peak flow > -270 L/min underwent 5 consecutive days of 5 cough augmentation sessions; cough peak flow during exsufflation and the total insufflation volume (TIV) during insufflation were measured. Only MI-E was administered on days 1 and 5, whereas on days 2-4 one MI-E-only session followed by 3 MI-E and manually assisted cough sessions was implemented followed by a fifth MI-E-only session. The cumulative and carry-over effects of increasing treatment sessions and any associated factor on cough peak flow during MI-E-assisted coughing were assessed using a linear mixed model (LMM) with repetitive air-flow measurements within the same participants. Results: No cumulative or carry-over effects of manually assisted cough and MI-E were shown with the accumulation of treatment days or sessions. The LMM confirmed that using manually assisted cough (-0.283 L/s, P < .001), TIV (-0.045 L/s, P = .002), and the individual manually assisted cough variance (-0.022 L/s, P = .01) significantly influenced cough peak flow. Estimated mean cough peak flows for MI-E with manually assisted cough and MI-E alone were -4.006 L/s (95% CI -4.237 to -3.775) and -3.723 L/s (95% CI -3.953 to -3.492), respectively, surpassing the initial voluntary cough peak flow without MI-E assistance (-1.65 ± 0.53 L/s). CONCLUSIONS: The use of manually assisted cough and amount of TIV correlated with improved cough peak flow, emphasizing the importance of adequate in-expiratory support. No carry-over effect was associated with using manually assisted cough, highlighting the need to combine MI-E with manually assisted cough for each MI-E treatment to achieve optimal cough effectiveness. [ABSTRACT FROM AUTHOR]

BACKGROUND: Mechanical insufflation-exsufflation (MI-E) applied through the endotracheal tube (ETT) can effectively eliminate airway secretions in intubated patients. However, the effect of the interface (ETT vs face mask) on expiratory air flow generated by MI-E has not been investigated. This study aimed to investigate the effect of the ETT on peak expiratory flow (PEF) along with other associated factors that could influence PEF generated by MI-E. METHODS: Intubated participants received 2 sessions of MI-E via ETT therapy per d for 2 consecutive days. One MI-E session consisted of 5 sets of either constant (+40/-40 cm H2O) or incremental (+30/-30 to +50/-50 cm H2O) pressure applications. Following extubation, MI-E sessions were repeated using face mask. Expiratory air flow during MI-E therapy was continuously measured, and every PEF during each application was analyzed using linear mixed-effect and generalized linear mixed models. RESULTS: A total of 12 participants (9 [75.0%] men; mean [SD] age, 74.0 [10.2] y) completed all MI-E sessions with both ETT and face mask interfaces. The PEF generated during MI-E treatment was influenced by the interface (ETT vs face mask), pressure gradient, and number of session repetitions. Adjusted mean PEF values for MI-E via ETT and face mask at +40/-40 cm H2O were -2.521 and -3.114 L/s, respectively, and -2.956 and -3.364 L/s at +50/-50 cm H2O, respectively. At a pressure gradient of +40/-40 cm H2O, only 172 of 528 MI-E trials via ETT (32.6%) achieved a PEF faster than -2.7 L/s, whereas 304 of 343 MI-E trials via face mask (88.6%) exceeded PEF < -2.7 L/s. CONCLUSIONS: MI-E via ETT generated slower PEF than via face mask, suggesting that a higher-pressure protocol should be prescribed for intubated patients. An insufflation-exsufflation pressure up to +50/-50 cm H2O could be considered to produce a PEF faster than 2.7 L/s, and the applications were safe and feasible for subjects on invasive mechanical ventilation. [ABSTRACT FROM AUTHOR]

Vanishing white matter (VWM) disease is an autosomal recessive disorder that affects the central nervous system of a patient, and is caused by the development of pathogenic mutations in any of the EIF2B1-5 genes. Any dysfunction of the EIF2B1-5 gene encoded eIF2B causes stress-provoked episodic rapid neurological deterioration in the patient, followed by a chronic progressive disease course. We present the case of a patient with an infantile-onset VWM with the pre-described specific clinical course, subsequent neurological aggravation induced by each viral infection, and the noted consequent progression into a comatose state. Although the initial brain magnetic resonance imaging did not reveal specific pathognomonic signs of VWM to distinguish it from other types of demyelinating leukodystrophy, the next-generation sequencing studies identified heterozygous missense variants in EIF2B3, including a novel variant in exon 7 (C706G), as well as a 0.008% frequency reported variant in exon 2 (T89C). Hence, the characteristic of unbiased genomic sequencing can clinically affect patient care and decision-making, especially in terms of the consideration of genetic disorders such as leukoencephalopathy in pediatric patients. [ABSTRACT FROM AUTHOR]