Music-Based Intervention Ameliorates Mecp2-Loss-Mediated Sociability Repression in Mice through the Prefrontal Cortex FNDC5/BDNF Pathway.

Patients with Rett syndrome (RTT) show severe difficulties with communication, social withdrawl, and learning. Music-based interventions improve social interaction, communication skills, eye contact, and physical skills and reduce seizure frequency in patients with RTT. This study aimed to investigate the mechanism by which music-based interventions compromise sociability impairments in mecp2 null/y mice as an experimental RTT model. Male mecp2 null/y mice and wild-type mice (24 days old) were randomly divided into control, noise, and music-based intervention groups. Mice were exposed to music or noise for 6 h/day for 3 consecutive weeks. Behavioral patterns, including anxiety, spontaneous exploration, and sociability, were characterized using open-field and three-chamber tests. BDNF, TrkB receptor motif, and FNDC5 expression in the prefrontal cortex (PFC), hippocampus, basal ganglia, and amygdala were probed using RT-PCR or immunoblotting. mecp2 null/y mice showed less locomotion in an open field than wild-type mice. The social novelty rather than the sociability of these animals increased following a music-based intervention, suggesting that music influenced the mecp2-deletion-induced social interaction repression rather than motor deficit. Mechanically, the loss of BDNF signaling in the prefrontal cortex and hippocampal regions, but not in the basal ganglia and amygdala, was compromised following the music-based intervention in mecp2 null/y mice, whereas TrkB signaling was not significantly changed in either region. FNDC5 expression in the prefrontal cortex region in mecp2 null/y mice also increased following the music-based intervention. Collective evidence reveals that music-based interventions improve mecp2-loss-induced social dysfunction. BDNF and FNDC5 signaling in the prefrontal cortex region mediates the music-based-intervention promotion of social interactions. This study gives new insight into the mechanisms underlying the improvement of social behaviors in mice suffering from experimental Rett syndrome following a music-based intervention.

The effectiveness of music therapy for individuals with Rett syndrome and their families.

BACKGROUND: Patients with Rett syndrome (RTT) present characteristic regression in communication and hand skills, which eventually leads to intellectual and physical disability. Moreover, caregivers of patients with RTT face stressors related to patients' medical and developmental concerns. Given the indications from case reports, this pilot study investigated the effectiveness of music therapy on RTT patients, as well as on parental stress for families of children with RTT. METHODS: Families in the study group were enrolled in a twice-weekly 120-minute music therapy program for 24 weeks (n = 11), whereas families in the control group did not receive music therapy (n = 12). Participants were administered the Vineland Adaptive Behavior Scales, Rett Syndrome Clinical Severity Scale, Rett Syndrome Motor Behavioral Assessment, and Parenting Stress Index for caregivers of RTT children before and after the music therapy program. RESULTS: Music therapy improved receptive language, verbal and non-verbal communication skills, and social interaction for RTT patients. In addition, purposeful hand function, breathing patterns, and eye contact were significantly improved. Of note, music therapy also decreased the frequency of epileptic seizures. Lastly, caregivers in the study group exhibited significantly lower stress following the program. CONCLUSION: The 24-week music therapy program was effective in improving social interaction, communication skills, eye contact, hand function, and reducing seizure frequency among RTT patients. Additionally, music therapy was effective in relieving parenting stress, which may help healthcare providers initiate early intervention strategies that can prevent parenting stress and reduce the risk of depression.

Systematic review and meta-analysis of the diagnostic accuracy of the water swallow test for screening aspiration in stroke patients.

AIM: The aim of this study was to determine the diagnostic accuracy of the water swallow test for screening aspirations in stroke patients. BACKGROUND: The water swallow test is a simple bedside screening tool for aspiration among stroke patients in nursing practice, but results from different studies have not been combined before. DESIGN: A systematic review and meta-analysis was conducted to provide a synthetic and critical appraisal of the included studies. DATA SOURCES: Electronic literature in MEDLINE, EMBASE, CINAHL and other sources were searched systemically in this study. Databases and registers were searched from inception up to 30 April 2015. REVIEW METHODS: This systematic review was conducted using the recommendations from Cochrane Collaboration for Systematic Reviews of Diagnostic Test Accuracy. Bivariate random-effects models were used to estimate the diagnostic accuracy across those studies. The tool named Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) was used to evaluate the quality of the studies. RESULTS: There were 770 stroke patients in the 11 studies for the meta-analysis. The water swallow test had sensitivities between 64-79% and specificities between 61-81%. Meta-regression analysis indicated that increasing water volume resulted in higher sensitivity but lower specificity of the water swallow test. CONCLUSIONS: This systematic review showed that the water swallow test was a useful screening tool for aspiration among stroke patients. The test accuracy was related to the water volume and a 3-oz water swallow test was recommended for aspiration screening in stroke patients.

Therapeutic effects of extracorporeal shock wave therapy on patients with spastic cerebral palsy and Rett syndrome: clinical and ultrasonographic findings.

BACKGROUND: Extracorporeal shock wave therapy (ESWT) is reportedly effective for improving spasticity and motor function in children with cerebral palsy (CP). Because late-stage Rett syndrome has a similar presentation, this study aimed to investigate the effects of ESWT on these two diseases. MATERIAL AND METHODS: Patients diagnosed with spastic CP and Rett syndrome received 1500 impulses of ESWT at 4 Hz and 0.1 mJ/mm2, on their spastic legsonce weekly for a total of 12 weeks. Outcomes were assessed before and 4 and 12 weeks after ESWT. Clinical assessments included the Modified Ashworth Scale (MAS), passive range of motion (PROM), and Gross Motor Function Measure 88 (GMFM-88). Ultrasonographic assessments included muscle thickness, acoustic radiation force impulse (ARFI), and strain elastography. RESULTS: Fifteen patients with CP and six with Rett syndrome were enrolled in this study. After ESWT, patients with CP showed significant clinical improvement in the MAS (P = 0.011), ankle PROM (P = 0.002), walking/running/jumping function (P = 0.003), and total function (P < 0.001) of the GMFM-88. The patients with Rett syndrome showed improved MAS scores (P = 0.061) and significantly improved total gross motor function (P = 0.030). Under ARFI, patients with CP demonstrated decreased shear wave speed in the gastrocnemius medial head (P = 0.038). Conversely, patients with Rett syndrome show increased shear-wave speeds after ESWT. CONCLUSION: Our study provides evidence that a weekly course of low-dose ESWT for 12 weeks is beneficial for children with both CP and Rett syndrome, with the clinical effects of reducing spasticity and improving the gross motor function of the lower limbs. The ARFI sonoelastography reveals improvement of muscle stiffness in patients with CP after ESWT, but deteriorated in patients with Rett syndrome. The diverse therapeutic response to ESWT may be caused by the MECP2 mutation in Rett syndrome, having a continuous impact and driving the pathophysiology differently as compared to CP, which is secondary to a static insult. Trial registration IRB 201700462A3. Registered 22March 2017, https://cghhrpms.cgmh.org.tw/HRPMS/Default.aspx .

Relationship between the head deviation angle and hemineglect in patients with right hemisphere stroke.

BACKGROUND: Patients with hemineglect have been reported to have abnormal head posture. We attempted to determine the extent to which the angles are correlated with the severity of hemineglect. METHODS: In a prospective, single-center, cross-sectional study on 40 patients with single right hemisphere stroke, we evaluated left hemineglect severity using the line bisection and line cancellation tests. Head deviation angles were measured at admission and discharge using a head device containing 3 graduated discs that determined the angles of head deviation in the sagittal, coronal and transverse planes. RESULTS: Head angles in the transverse and coronal planes differed significantly between the left hemineglect and non-hemineglect groups. Scores obtained from the line bisection and line cancellation tests correlated with these angles. Furthermore, improvement in left hemineglect after rehabilitation training corresponded to a reduction in the angles. CONCLUSION: Stroke patients with hemineglect exhibited significant head deviations in the transverse and coronal planes. Deviation angles correlated with hemineglect test scores.

Effectiveness of ultrasound-guided vs direct approach corticosteroid injections for carpal tunnel syndrome: A double-blind randomized controlled trial.

OBJECTIVE: To compare the outcomes of ultrasound-guided vs direct approach corticosteroid injection in patients with idiopathic carpal tunnel syndrome. METHODS: A double-blind randomized controlled study. Wrists affected by carpal tunnel syndrome were randomized to the ultrasound-guided (n = 22 wrists) or direct approach injection group (n = 17 wrists) before receiving 1 ml Betamethasone. Outcome measures were physical findings and electrodiagnostic parameters assessed at 1, 3 and 6 months after injection. Complications were also recorded. RESULTS: Both groups showed improvement through-out the follow-up period after injections, in physical findings and in most electrodiagnostic parameters (all p<0.05). The ultrasound-guided injection group showed greater improvements in the Semmes-Weinstein Monofilament test result (p = 0.004), sensory nerve conduction velocity (p = 0.038), and digit-4 comparison study result (p = 0.046). Three wrists with weakness were found in the direct approach injection group, yet none were noted in the ultrasound-guided injection group (p=0.040). CONCLUSION: Both ultrasound-guided and direct approach corticosteroid injection protocols improved clinical symptoms and signs, physical function, and most electrodiagnostic parameters of patients with carpal tunnel syndrome throughout the follow-up period. However, the ultrasound-guided injection group showed greater improvements in the Semmes-Weinstein Monofilament test, sensory nerve conduction velocity, and digit-4 comparison study.

Inspiratory muscle training in stroke patients with congestive heart failure: A CONSORT-compliant prospective randomized single-blind controlled trial.

BACKGROUND: Cardiopulmonary function can be adversely affected after a cerebrovascular accident in patients with congestive heart failure (CHF). The aim of this study was to investigate the efficacy and feasibility of inspiratory muscle training (IMT) for stroke patients with CHF. METHODS: A prospective randomized single-blind controlled trial was conducted in a single tertiary medical center in southern Taiwan between May 2011 and July 2015. Forty-one patients were enrolled, of whom 21 completed the study (IMT group n = 11 and control group n = 10). Both groups participated in a conventional stroke rehabilitation program. Patients in the IMT group received an additional IMT program beginning with an intensity of 30% maximal inspiratory pressure (MIP), then increased by 2cmH2O each week for 30 minutes daily for at least 5 days a week for 10 weeks. MIP, maximal expiratory pressure, spirometry, resting oxyhemoglobin saturation, modified Borg Scale, Fatigue Assessment Scale, and Barthel Index were assessed in each patient. RESULTS: There were significant differences from baseline in MIP (P = 0.008), percent predicted forced vital capacity (P = 0.033), forced expiratory volume in 1 second (FEV1) (P = 0.008), percent predicted FEV1 (P = 0.008), and Barthel Index (P = 0.012) in the IMT group, and Barthel Index (P = 0.027) in the control group. There were significant differences between groups in MIP (20.91 ±â€Š19.73 vs -9.00 ±â€Š26.01, adjusted P value = 0.023) and Barthel Index (24.55 ±â€Š22.30 vs 7.50 ±â€Š8.25, adjusted P value = 0.044). CONCLUSION: The 10-week IMT was feasible and effective in improving inspiratory force and activities of daily living for the stroke patients with CHF.

Efficacy of constraint-induced therapy on functional performance and health-related quality of life for children with cerebral palsy: a randomized controlled trial.

To better generalize training effects to the context of daily living, home-based constraint-induced therapy has been proposed. Therapeutic success of constraint-induced therapy is limited as to whether the improvements in functional performance can be transferred to quality of life. This randomized controlled trial aimed to investigate the efficacy of home-based constraint-induced therapy on functional performance and health-related quality of life. Twenty-two children with spastic unilateral cerebral palsy (6-8 years, 10 boys) were randomly assigned to receive constraint-induced therapy or traditional rehabilitation. Home-based constraint-induced therapy had immediate and maintaining effects on motor efficacy and functional performance and induced greater gains in health-related quality of life in the long run than in the short term. The home-based constraint-induced therapy protocol (relatively moderate intensity and shortened constraint time), which might balance the effectiveness and compliance of participants and caregivers, may be an effective alternative to conventional constraint-induced therapy.

Effect of neuromuscular electrical stimulation in a patient with Sjogren's syndrome with dysphagia: a real time videofluoroscopic swallowing study.

Severe dysphagia in a 54 year-old woman with Sjogren's syndrome with involvement of multiple cranial nerves significantly improved after treatment with neuromuscular electrical stimulation (NMES) in combination with a swallowing rehabilitation program. The swallowing response was assessed in real time using a videofluoroscope. Immediate improvement in the tongue retraction force, clearing of the valleculae, increase in laryngeal elevation and shortening of pharyngeal transit time were noted during stimulation. The patient returned to independent oral feeding after 46 sessions of NMES. After follow-up for 1 year, we found that the patient maintained adequate oral feeding and did not show signs of pulmonary complications.