Impact of dance classes on motor and cognitive functions and gut microbiota composition in multiple sclerosis patients: Randomized controlled trial.

Evidence suggests that multiple sclerosis (MS) induces a decline in motor and cognitive function and provokes a shift in gut microbiome composition in patients. Therefore, the aim of the study was to explore the effect of dance classes on the motor and cognitive functions and gut microbiota composition of MS patients. In this randomized controlled trial, 36 patients were randomly divided into two groups: the experimental group (n = 18) and the passive control group (n = 18). Supervised rock and roll and sports dance classes were performed for 12 weeks at a frequency of two times a week. Before and after the intervention, fecal samples were taken and the motor and cognitive function assessments were completed. Fecal microbiota were categorized using primers targeting the V3-V4 region of 16S rDNA. Our results revealed significant differences in mobility performance (T25-FWT), attention and working memory (TMT B), and finger dexterity (9-HPT) within the experimental group. Furthermore, we reported favorable shifts in gut microbial communities (an increase in Blautia stercoris and a decrease in Ruminococcus torques) within the experimental group. In conclusion, our randomized control trial on the effects of 12-week dance classes in MS patients found significant improvements in motor and cognitive functions, with further moderate influence on gut microbiota composition.

Use of cortical hemodynamic responses in digital therapeutics for upper limb rehabilitation in patients with stroke.

BACKGROUND: Stroke causes long-term disabilities, highlighting the need for innovative rehabilitation strategies for reducing residual impairments. This study explored the potential of functional near-infrared spectroscopy (fNIRS) for monitoring cortical activation during rehabilitation using digital therapeutics. METHODS: This cross-sectional study included 18 patients with chronic stroke, of whom 13 were men. The mean age of the patients was 67.0 ± 7.1 years. Motor function was evaluated through various tests, including the Fugl-Meyer assessment for upper extremity (FMA-UE), grip and pinch strength test, and box and block test. All the patients completed the digital rehabilitation program (MotoCog®, Cybermedic Co., Ltd., Republic of Korea) while being monitored using fNIRS (NIRScout®, NIRx Inc., Germany). Statistical parametric mapping (SPM) was employed to analyze the cortical activation patterns from the fNIRS data. Furthermore, the K-nearest neighbor (K-NN) algorithm was used to analyze task performance and fNIRS data to classify the severity of motor impairment. RESULTS: The participants showed diverse task performances in the digital rehabilitation program, demonstrating distinct patterns of cortical activation that correlated with different motor function levels. Significant activation was observed in the ipsilesional primary motor area (M1), primary somatosensory area (S1), and contralateral prefrontal cortex. The activation patterns varied according to the FMA-UE scores. Positive correlations were observed between the FMA-UE scores and SPM t-values in the ipsilesional M1, whereas negative correlations were observed in the ipsilesional S1, frontal lobe, and parietal lobe. The incorporation of cortical hemodynamic responses with task scores in a digital rehabilitation program substantially improves the accuracy of the K-NN algorithm in classifying upper limb functional levels in patients with stroke. The accuracy for tasks, such as the gas stove-operation task, increased from 44.4% using only task scores to 83.3% when these scores were combined with oxy-Hb t-values from the ipsilesional M1. CONCLUSIONS: The results advocated the development of tailored digital rehabilitation strategies by combining the behavioral and cerebral hemodynamic data of patients with stroke. This approach aligns with the evolving paradigm of personalized rehabilitation in stroke recovery, highlighting the need for further extensive research to optimize rehabilitation outcomes.

Sex-specific associations of a ferroalloy metal mixture with motor function in Italian adolescents.

Background: Motor function is critical for children's health, yet remains an understudied neurodevelopmental domain. Exposure to metals has been linked with motor function, but no study has examined the joint effects of metal mixtures. Methods: We evaluated cross-sectional associations between a metal mixture and motor function among 569 adolescents (10-14 years old) living near the ferroalloy industry. Concentrations of blood lead, hair manganese, hair copper, and hair chromium were quantified using inductively coupled plasma mass spectrometry. Neuropsychologists administered multiple fine motor function assessments: pursuit aiming, finger tapping, visual reaction time (VRT), and subtests from the Luria Nebraska battery. We estimated associations between motor function and the metal mixture using quantile-based g-computation and multivariable linear regression, adjusting for child age, sex, and socioeconomic status. We explored sex-specific associations in stratified models. Results: Associations between the metal mixture and motor function were mostly null but were modified by sex. We observed a beneficial association among females: a quartile increase in all metals in the mixture was associated with a 2.6% faster average response time on the VRT (95% confidence interval [CI] = -4.7%, -0.5%), driven by Cu and Cr. In contrast, this association was adverse among males (ß = 1.5% slower response time [95% CI = -0.7%, 3.9%]), driven by Cu and Mn. Conclusions: Results suggest that males may be more susceptible to the adverse effects of metal exposure on motor function during adolescence than females. Future studies, particularly prospective study designs, are warranted to further understand the associations of metal mixtures with motor function.

Direct Photobiomodulation Therapy on the Sciatic Nerve Significantly Attenuates Acute Nociceptive Sensitivity Without Affecting Motor Output.

OBJECTIVES: Pharmacologic pain treatments lack specific targeting and often produce unwanted side effects (eg, addiction, additional hyperalgesia). We previously established that the direct application of laser irradiation (direct photobiomodulation [PBM]) of the sural nerve reduces thermal hypersensitivity in a rodent model of chronic pain, but not mechanical hypersensitivity. These observations were consistent with a selective reduction in the small-diameter fiber contribution to electrophysiologically measured evoked response after direct PBM of a sensory nerve (saphenous). However, to our knowledge, direct application of laser irradiation has never been performed in an animal model of acute nociceptive pain or on a mixed nerve in which sensory and motor outcomes can be observed. MATERIALS AND METHODS: In this study, we describe the effects of direct application of laser irradiation (808 nm, 60 mW, 4 minutes) on a mixed nerve (sciatic nerve) in an acute nociceptive pain model (intradermal capsaicin injection) in rats over the course of two weeks. To investigate whether laser irradiation of a mixed nerve alters motor function, in separate experiments, we applied laser irradiation to the sciatic nerve (using the same parameters as in the chronic pain experiments), and force generation of the gastrocnemius was measured. RESULTS: Capsaicin-induced hypersensitivities to mechanical (pin prick) and thermal (Hargreaves) noxious stimuli, associated with Aδ- and C-fibers, showed a maximal reduction of 70% and 56.2%, respectively, by direct PBM, when compared with a control group (vehicle injection, no PBM) on the same day. This reduction was determined to be significant using a mixed-design analysis of variance with a p value < 0.05. Force generation remained unchanged for up to 120 minutes after laser irradiation. In summary, direct PBM selectively inhibits C- and Aδ-fiber transmission while leaving Aɑ-, Aß-, and motor-fiber activity intact. CONCLUSIONS: These results, in conjunction with our previous analyses of laser irradiation effects on the sural nerve in a chronic spared nerve injury pain model, suggest that direct PBM is a promising candidate for treating pain induced by small-diameter fiber activity.

Implications of the diagnosis of locomotive syndrome stage 3 for long-term care.

Objectives: Locomotive syndrome stage 3 (LS3), which has been established recently, may imply a greater need for care than LS stage 0 (LS0), LS stage 1 (LS1), and LS stage 2 (LS2). The relationship between LS3 and long-term care in Japan is unclear. Therefore, this study aimed to examine this relationship. Methods: A total of 531 patients (314 women and 217 men; mean age, 75 years) who were not classified as requiring long-term care and underwent musculoskeletal examinations in 2012 were grouped according to their LS stage. Group L comprised patients with LS3 and Group N comprised those with LS0, LS1, and LS2. We compared these groups according to their epidemiology results and long-term care requirements from 2013 to 2018. Results: Fifty-nine patients (11.1%) were diagnosed with LS3. Group L comprised more patients (50.8%) who required long-term care than Group N (17.8%) (P < 0.001). Group L also comprised more patients with vertebral fractures and knee osteoarthritis than Group N (33.9% vs 19.5% [P = 0.011] and 78% vs 56.4% [P < 0.001], respectively). A Cox proportional hazards model and Kaplan-Meier analysis revealed a significant difference in the need for nursing care between Groups L and N (log-rank test, P < 0.001; hazard ratio, 2.236; 95% confidence interval, 1.451-3.447). Conclusions: Between 2012 and 2018, 50% of patients with LS3 required nursing care. Therefore, LS3 is a high-risk condition that necessitates interventions. Approaches to vertebral fractures and osteoarthritis of the knee could be key.

Correlation between motor function and health-related quality of life in early to mid-stage patients with Parkinson disease: a cross-sectional observational study.

Aim: To investigate the correlation between motor function and health-related quality of life (HrQOL) in early to mid-stage patients with Parkinson disease (PwP). Methods: This cross-sectional study recruited PwP from April 2020 to December 2023 at the outpatient clinic of Peking Union Medical College Hospital in Beijing, China. The motor symptoms were assessed using Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part 3. Balance function was evaluated using the Berg Balance Scale (BBS), and the risk of fall using Timed Up-and-Go test (TUG), and Five Times Sit-to-Stand test (FTSST). Freezing of gait questionnaire (FOGQ) was used to evaluate the severity of gait. The Intelligent Device for Energy Expenditure and Physical Activity (IDEEA) recorded gait cycle parameters, and the isokinetic dynamometer measured muscle strength. The Parkinson's Disease Questionnaire-39 (PDQ-39) was used to measure HrQOL. All assessments were tested during the on state. Spearman correlation was conducted to evaluate the correlation between motor function and HrQOL. Results: 243 patients with mean age of 69.33 years were enrolled. The PDQ-39 score was strongly correlated with FOG in H&Y stage III (r = 0.653, p < 0.001) and moderately correlated in H&Y stage I (r = 0.471, p < 0.001) and H&Y stage II (r = 0.386, p < 0.001). Furthermore, the FOG was strongly correlated with mobility domain at H&Y stage III (r = 0.694, p < 0.001) and moderately correlated at H&Y stage I (r = 0.431, p < 0.001) and H&Y stage II (r = 0.434, p < 0.001). All motor function scores were correlated with PDQ-39 scores at H&Y stage III (p < 0.05). Conclusion: Motor function correlated with HrQOL in early to mid-stage PwP, and FOG was the main factor, especially affecting mobility, activities of daily life and communication. HrQOL in patients at different disease stages were variously affected by motor function, and HrQOL and multiple dimensions was significantly associated with motor function in patients at H&Y stage III.

Association between the Hopkins Falls grading scale and motor function tests in patients with multiple sclerosis.

Introduction: One of the main complications of multiple sclerosis (MS) is imbalance and walking problems that can lead to falls. This study investigated the association between a fall measurement scale called the Hopkins Falls Grading Scale (HFGS) and motor function tests in patients with MS. Material and Methods: This cross sectional study was conducted using convenience sampling on 85 patients referred to the MS Association of Mashhad, Iran, in 2023. The HFGS examined falls during the past year and divided them into 4 degrees, and the function test included the timed 25 foot walk (T25FW) test and the timed up and go (TUG) test. Kruskal-Wallis test and Spearman's correlation coefficient were used for data analysis. Results: A statistically significant association was obtained between HFGS and functional tests (T25FW and TUG) (for both P < 0.0001). A significant association was observed between the variables of age (P = 0.006), duration of the disease (P = 0.03), the use of mobility devices (P = 0.05), and HFGS. Conclusion: Considering the association between HFGS and motor function tests in MS patients, clinical experts should pay attention to patients who have slower movement and evaluate them in terms of falling status when performing motor function tests.

The effect of body weight-supported Tai Chi Yunshou on upper limb motor function in stroke survivors based on neurobiomechanical analysis: a four-arm, parallel-group, assessors-blind randomized controlled trial protocol.

Introduction: A series of functional disorders commonly occur after stroke, of which upper limb dysfunction is the most difficult to recover. The upper limb rehabilitation effect of Tai Chi Yunshou(TCY) in the later stage of stroke has been confirmed by research. Body weight support-Tai Chi Yunshou (BWS-TCY) is based on TCY exercise and robotic exoskeletons offers most flexibility in deweighting and control strategy. This study is aimed to explore the effect of BWS-TCY on upper limb motor function in stroke based on neurobiomechanics. Methods and analysis: A single-blind randomized controlled trial will be conducted on 36 stroke survivors who will be randomly assigned to three groups: experimental group, control group A and control group B. In addition, 12 healthy elderly people will be recruited into the healthy control group. Those in the experimental group will receive 20 min of CRT and 20 min of BWS-TCY training, while participants in the control group A will receive 20 min of CRT and 20 min of Robot-assisted training. Participants in the control group B will undergo 40 min of Conventional rehabilitation training (CRT) daily. All interventions will take place 5 days a week for 12 weeks, with a 12-week follow-up period. No intervention will be carried out for the healthy control group. Upper limb function will be assessed before and after the intervention using various rating scales (Fugl-Meyer Assessment, Wolf Motor Function Test, etc.), as well as neurobiomechanical analyses (surface electromyography, functional near-infrared brain function analysis system, and Xsens maneuver Capture System). Additionally, 10 healthy elderly individuals will be recruited for neurobiomechanical analysis, and the results will be compared with those of stroke survivors. Discussion: The results of this study will offer initial evidence on the effectiveness and feasibility of BWS-TCY as an early intervention for stroke rehabilitation. Positive findings from this study could contribute to the development of guidelines for the use of BWS-TCY in the early stages of stroke. Ethics and dissemination: This study has been approved by the Research Ethics Committees of the seventh People's Hospital Affiliated to Shanghai University of Traditional Chinese Medicine (Study ID: 2022-7th-HIRB-022). The results of the study will be published in a peer-reviewed journal and presented at scientific conferences. Clinical trial registration: https://clinicaltrials.gov/, ChiCTR 2200063150.

Repetitive transcranial magnetic stimulation promotes motor function recovery in mice after spinal cord injury via regulation of the Cx43-autophagy loop.

Spinal cord injury (SCI) is a severe condition with an extremely high disability rate. It is mainly manifested as the loss of motor, sensory and autonomic nerve functions below the injury site. High-frequency transcranial magnetic stimulation, a recently developed neuromodulation method, can increase motor function in mice with spinal cord injury. This study aimed to explore the possible mechanism by which transcranial magnetic stimulation (TMS) restores motor function after SCI. A complete T8 transection model of the spinal cord was established in mice, and the mice were treated daily with 15 Hz high-frequency transcranial magnetic stimulation. The BMS was used to evaluate the motor function of the mice after SCI. Western blotting and immunofluorescence were used to detect the expression of Connexin43 (CX43) and autophagy-related proteins in vivo and in vitro, and correlation analysis was performed to study the relationships among autophagy, CX43 and motor function recovery after SCI in mice. Western blotting was used to observe the effect of magnetic stimulation on the expression of mTOR pathway members. In the control group, the expression of CX43 was significantly decreased, and the expression of microtubule-associated protein 1 A/1b light chain 3 (LC3II) and P62 was significantly increased after 4 weeks of spinal cord transection. After high-frequency magnetic stimulation, the level of CX43 decreased, and the levels of LC3II and P62 increased in primary astrocytes. The BMS of the magnetic stimulation group was greater than that of the control group. High-frequency magnetic stimulation can inhibit the expression of CX43, which negatively regulates autophagic flux. HF-rTMS increased the expression levels of mTOR, p-mTOR and p-S6. Our experiments showed that rTMS can restore hindlimb motor function in mice after spinal cord injury via regulation of the Cx43-autophagy loop and activation of the mTOR signalling pathway.

Cytokines in cerebrospinal fluid as a prognostic predictor after treatment of nusinersen in SMA patients.

OBJECTIVES: Recent studies have suggested that neuroinflammation may play a role in the progression of spinal muscular atrophy (SMA), and this may influence the efficacy of antisense oligonucleotide treatment. This study explored the biomarkers associated with SMA and the efficacy of nusinersen therapy. METHODS: Fifteen patients with SMA were enrolled and their motor function (World Health Organization motor milestone, Hammersmith Functional Motor Scale Expanded (HFMSE), and Revised Upper Limb Module [RULM] scores, and 6-minute walking test) was evaluated before, during (63 days), and after (6 months) nusinersen treatment. The concentrations of monocyte chemoactive protein 1 (MCP1), tumour necrosis factor-alpha (TNF-α), and interleukin (IL)-10 in the cerebrospinal fluid were measured at the indicated time points, and their correlations with motor function were analysed. RESULTS: A significant increase in MCP1 was observed after 6 month's treatment compared with that before treatment, while TNF-α gradually decreased over the course of treatment. IL-10 levels were negatively correlated with HFMSE scores before treatment, and reductions in IL-10 levels were correlated with improvements in RULM scores. CONCLUSIONS: This study suggests that neuroinflammation may be associated with the severity of SMA and with the therapeutic effects of nusinersen, which could have clinical implications in the treatment of SMA.

Exoskeleton-assisted upper limb rehabilitation after stroke: a randomized controlled trial.

OBJECTIVES: The upper-limb exoskeleton training program which is repetetive and task-specific therapy can improve motor functions in patients with stroke. To compare the effect of an upper-limb exoskeleton training program with Bobath concept on upper limb motor functions in individuals with chronic stroke. METHODS: Participants were randomly assigned to exoskeleton group (EG, n = 12) or to Bobath group (BG, n = 12). Interventions were matched in terms of session duration and total number of sessions and performed 2 times per week for 6-weeks. Primary outcome was Fugl-Meyer-Upper Extremity (FMA-UE). Secondary outcomes were Modified Ashworth Scale (elbow and wrist flexor muscles), Motor Activity Log-30 which is consist of two parts as an amount of use (AOU) and quality of movement (QOM), and The Nottingham Extended Activities of Daily Living (NEADL) index. RESULTS: After 12-sessions of training, the mean (SD) FMA-UE score increased by 5.7 (2.9) in the EG, and 1.9 (1.5) points in the BG (p < .05). In total, 40% of participants (5/12) demonstrated a clinically meaningful improvement (≥5.25 points) in the FM-UE, while none of the participants reached MCID score in the bobath group. Changes in the AOU, QOM, and NEADL were significantly larger in the EG compared to BG (p < .05). 7/12 (58.33%) of participants for AOU and 5/12 (42%) of participants for QOM in the EG showed that clinically meaningful change. 5/12 of participants (42%) in the EG demonstrated ≥4.9-point increase in NEADL score. DISCUSSION: High-intensity repetitive arm and hand exercises with an exoskeleton device was safe and feasible. Exoskeleton-assisted training demonstrated significant benefits in improving upper limb functions and quality of life in individuals after stroke.

Gait Reconstruction Strategy Using Botulinum Toxin Therapy Combined with Rehabilitation.

Numerous studies have established a robust body of evidence for botulinum toxin A (BoNT-A) therapy as a treatment for upper motor neuron syndrome. These studies demonstrated improvements in spasticity, range of joint motion, and pain reduction. However, there are few studies that have focused on improvement of paralysis or functional enhancement as the primary outcome. This paper discusses the multifaceted aspects of spasticity assessment, administration, and rehabilitation with the goal of optimising the effects of BoNT-A on lower-limb spasticity and achieving functional improvement and gait reconstruction. This paper extracts studies on BoNT-A and rehabilitation for the lower limbs and provides new knowledge obtained from them. From these discussion,, key points in a walking reconstruction strategy through the combined use of BoNT-A and rehabilitation include: (1) injection techniques based on the identification of appropriate muscles through proper evaluation; (2) combined with rehabilitation; (3) effective spasticity control; (4) improvement in ankle joint range of motion; (5) promotion of a forward gait pattern; (6) adjustment of orthotics; and (7) maintenance of the effects through frequent BoNT-A administration. Based on these key points, the degree of muscle fibrosis and preintervention walking speed may serve as indicators for treatment strategies. With the accumulation of recent studies, a study focusing on walking functions is needed. As a result, it is suggested that BoNT-A treatment for lower limb spasticity should be established not just as a treatment for spasticity but also as a therapeutic strategy in the field of neurorehabilitation aimed at improving walking function.

Effects of robot rehabilitation on the motor function and gait in children with cerebral palsy: a systematic review and meta-analysis.

This study was to determine the effects of robot rehabilitation on motor function and gait in children with cerebral palsy (CP) and the effect of robot type. Inclusion criteria were children with any type of CP, robot rehabilitation studies, non-robot rehabilitation comparison groups, outcomes related to motor function and gait, and randomized controlled trials. PubMed, Embase, Cochrane Library, CINAHL, and Web of Science databases were searched. Risk of bias was assessed using physiotherapy evidence database. Seven studies with a total of 228 participants were selected. Motor function was significantly improved in three studies comparing robot rehabilitation and control groups (standard mean difference [SMD], 0.79; 95% confidence intervals [CIs], 0.34-1.24; I 2=73%). Gait was not significantly improved in five studies comparing robot rehabilitation and control groups (SMD, 0.27; 95% CI, -0.09 to 0.63; I 2=45%). When comparing effects by robot type, robotic-assisted gate training (RAGT) showed significant improvements in both motor function (SMD, 0.89; 95% CI, 0.36-1.43; I 2=77%) and gait (SMD, 0.62; 95% CI, 0.12-1.11; I 2=44%). Robot rehabilitation effectively improved motor function, and among the robot types, RAGT was found to be effective in improving motor function and gait.

Stepping towards health: a scoping review of square-stepping exercise protocols and outcomes in older adults.

BACKGROUND: Square-Stepping Exercise (SSE) is a type of physical-cognitive exercise. Such exercise has been used as an exercise approach in different studies with older adults. This scoping review provides an overview of the protocols and outcomes of studies employing the SSE in older adults. METHODS: We searched in the PubMed, CINAHL, Scopus, CABI Global Health, and Web of Science databases for articles published between 2006 (first research article published on SSE) to December 2023 that met a robust inclusion criterion. The search yielded 424 articles, and after inclusion criteria being applied, 37 articles were included in the final analysis. RESULTS: A total of 37 studies were included in the final analysis. Thirty-three out of the 37 studies focused on apparently healthy older adults, while four were conducted with older adults with neurological disease (i.e., multiple sclerosis, Parkinson's disease, and stroke). Most studies (n = 25) adopted an experiment (i.e., randomized controlled trial) or quasi-experimental approach, while 12 were classified as non-randomized (i.e., cross-sectional, mixed methods). The studies were conducted in different parts of the globe and adopted three major formats of intervention delivery, namely in-person, online, and home-based. Frequency, SSE session duration and intervention length significantly varied among studies, and reported outcomes were in the domains of physical and cognitive function. CONCLUSION: This review comprehensively described the characteristics of 37 studies employing SSE in apparently healthy older adults and older adults with neurological diseases. The findings demonstrated that SSE has been used by researchers across the globe, adopting a variety of forms of delivery, and to particularly improve physical and cognitive function of different segments of the older adult population. The review further identified important gaps in research, including the restricted outcomes, and the lack of studies combining SSE with more traditional exercise modalities to address potential combinatory effects.

Development of a mouse model of chronic ventral spinal cord compression: Neurobehavioral, radiological, and pathological changes.

Objectives: The main objective of this study was to establish a mouse model of spinal ligament ossification to simulate the chronic spinal cord compression observed in patients with ossification of the posterior longitudinal ligament (OPLL). The study also aimed to examine the mice's neurobiological, radiological, and pathological changes. Methods: In the previous study, a genetically modified mouse strain was created using Crispr-Cas9 technology, namely, Enpp1 flox/flox /EIIa-Cre (C57/B6 background), to establish the OPLL model. Wild-type (WT) mice without compression were used as controls. Functional deficits were evaluated through motor score assessment, inclined plate testing, and gait analysis. The extent of compression was determined using CT imaging. Hematoxylin and eosin staining, luxol fast blue staining, TUNEL assay, immunofluorescence staining, qPCR, and Western blotting were performed to evaluate levels of apoptosis, inflammation, vascularization, and demyelination in the study. Results: The results demonstrated a gradual deterioration of compression in the Enpp1 flox/flox /EIIa-Cre mice group as they aged. The progression rate was more rapid between 12 and 20 weeks, followed by a gradual stabilization between 20 and 28 weeks. The scores for spinal cord function and strength, assessed using the Basso Mouse Scale and inclined plate test, showed a significant decline. Gait analysis revealed a noticeable reduction in fore and hind stride lengths, stride width, and toe spread. Chronic spinal cord compression resulted in neuronal damage and activated astrocytes and microglia in the gray matter and anterior horn. Progressive posterior cervical compression impeded blood supply, leading to inflammation and Fas-mediated neuronal apoptosis. The activation of Bcl2 and Caspase 3 was associated with the development of progressive neurological deficits (p < 0.05). Conclusions: The study presents a validated model of chronic spinal cord compression, enabling researchers to explore clinically relevant therapeutic approaches for OPLL.

Long-term impact of nusinersen on motor and electrophysiological outcomes in adolescent and adult spinal muscular atrophy: insights from a multicenter retrospective study.

BACKGROUND: 5q spinal muscular atrophy (SMA) is a progressive autosomal recessive motor neuron disease. OBJECTIVE: We aimed to assess the effects of nusinersen on motor function and electrophysiological parameters in adolescent and adult patients with 5q SMA. METHODS: Patients with genetically confirmed 5q SMA were eligible for inclusion, and clinical data were collected at baseline (V1), 63 days (V4), 180 days (V5), and 300 days (V6). The efficacy of nusinersen was monitored by encompassing clinical assessments, including the Revised Upper Limb Module (RULM), Hammersmith Functional Motor Scale Expanded (HFMSE), 6-Minute Walk Test (6MWT), and percent-predicted Forced Vital Capacity in sitting position (FVC%) and Compound Muscle Action Potential (CMAP) amplitude. The patients were divided into "sitter" and "walker" subgroups according to motor function status. RESULTS: 54 patients were screened, divided into "sitter" (N = 22) and "walker" (N = 32), with the mean age at baseline of 27.03 years (range 13-53 years). The HFMSE in the walker subgroup increased significantly from baseline to V4 (mean change +2.32-point, P = 0.004), V5 (+3.09, P = 0.004) and V6 (+4.21, P = 0.005). The patients in both the sitter and walker subgroup had no significant changes in mean RULM between V1 and the following time points. Significant increases in CMAP amplitudes were observed in both upper and lower limbs after treatment. Also, patients with RULM ≥ 36 points showed significant CMAP improvements. Our analysis predicted that patients with CMAP amplitudes of trapezius ≥ 1.76 mV were more likely to achieve significant motor function improvements. CONCLUSIONS: Nusinersen effectively improves motor function and electrophysiological data in adolescent and adult patients with SMA. This is the first report on the CMAP amplitude changes in the trapezius after treatment in patients with SMA. The CMAP values effectively compensate for the ceiling effect observed in the RULM, suggesting that CMAP could serve as an additional biomarker for evaluating treatment efficacy.

Clinical profile and associated comorbidities of cerebral palsy in children visiting Orotta National Referral Hospital, Eritrea: a cross-sectional study.

BACKGROUND: Cerebral Palsy (CP) is one of the most common physical disabilities in children. This study aimed to explore the clinical spectrum of CP at Orotta National Referral and Teaching Hospital, including CP subtypes, gross motor function, patterns of associated comorbidities, and possible risk factors in children aged 2 to 12 years. METHODS: A hospital-based cross-sectional study was conducted from January to April 2022 in 153 children with suspected motor symptoms. The Surveillance of CP in Europe (SCPE) decision tree was used as an inclusion criteria guideline and the evaluation of the participants was done using a standardized questionnaire and clinical examination. Descriptive statistics, chi-square test, and logistic regression were employed to statistically analyze the data. RESULTS: Eighty-four children who fulfilled the clinical criteria were included in the study. The median age was 5-years [IQR: 3.8] with an equal distribution of males and females. Quadriplegic CP was the most common subtype (51.2%) followed by unilateral (hemiplegic) CP (22.6%), and dyskinetic CP (14.3%). Most children had severe gross motor impairment GMFCS level IV-V and females were almost three times more likely to have GMFCS level IV/V than males (AOR: 2.70; CI: 1.08-6.72; p-value = 0.033.) More than half (52.4%) of the neonates either did not cry within five minutes and/or needed breathing resuscitation, 55.3% had to be admitted to the NICU with a median of 5 days' hospital stay. Between the first week of birth and the first year of life, 28.6% had trouble feeding, 26.2% had an infection, 10.7% had difficulty breathing, 20.2% had seizures and 6% had jaundice. Feeding problems (64.3%), speech problems of some sort (91.7%), and epilepsy (46.4%) were the most commonly associated comorbidities with CP. CONCLUSIONS: The clinical profile of the CP patients was found to be dominated by the spastic subtype and moderate to severe disability. Since perinatal risk factors were found to be dominant, strengthening maternal and child healthcare systems is recommended to minimize incidents of preventable risk factors and the burden of the disability.

Analysis of the efficacy and adverse effects of nusinersen in the treatment of children with spinal muscular atrophy in China.

OBJECTIVE: This study was based on a retrospective clinical observational cohort study of a two-center application of nusinersen in China to evaluate the clinical efficacy and adverse effects of nusinersen in the treatment of SMA (spinal muscular atrophy) Types 1-3. METHODS: Clinical data from children with clinically and genetically confirmed 5qSMA from a double center in western China (the Second Affiliated Hospital of Xi'an Jiaotong University and the Second Hospital of West China of Sichuan University). All children were younger than 18 years of age. Patients were assessed for motor function and underwent blood and fluid tests before each nusinersen injection. RESULTS: At 14-month follow-up, 100% of children had improved their HFMSE (Hammersmith Functional Motor Scale Expanded) score, 83.6% had improved their CHOP INTEND (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders) score, and 66.6% had improved their RULM (Revised Upper Limb Module) score by ≥3 points from baseline, and their 6MWT (6-min walk test) was 216.00 ± 52.08 m longer than at baseline. The age of the child at the start of treatment was negatively correlated with the clinical efficacy of nusinersen; the younger the child, the better the response to treatment. No significant adverse effects affecting the treatment and quality of life of the child were observed during the treatment of SMA with nusinersen. CONCLUSION: This study concluded that nusinersen is clinically beneficial for children with SMA in western China, with mild adverse effects.

Effects of Cluster Nursing Strategy on Neurological and Motor Functions in Patients with Moderate to Severe Traumatic Brain Injury.

This study aimed to explore the effects of the cluster nursing strategy applied to traumatic brain injury (TBI) patients. Ninety-eight TBI patients admitted to the hospital were selected as the study subjects. They were randomized into two groups, the control group and the cluster group, with 49 cases in each group. The control group received routine nursing methods, while the cluster group received cluster nursing strategy. The intervention effects were compared between the two groups. After 3 months, the total occurrence of complications in the cluster group was significantly lower than that in the control group. Postintervention, the cluster group had a significantly lower National Institutes of Health Stroke Scale score and significantly higher Fugl-Meyer score and Loewenstein Occupational Therapy Cognitive Assessment score compared with the control group. The serum level of glial fibrillary acidic protein in the control group was significantly higher than that in the cluster group, while the serum level of brain-derived neurotrophic factor was significantly lower. The application of the cluster nursing strategy in the care of patients with TBI could effectively reduce the risk of complications and improve neurological, motor, and cognitive functions.

Distance-related functional reorganization predicts motor outcome in stroke patients.

BACKGROUND: Analyzing distance-dependent functional connectivity density (FCD) yields valuable insights into patterns of brain activity. Nevertheless, whether alterations of FCD in non-acute stroke patients are associated with the anatomical distance between brain regions remains unclear. This study aimed to explore the distance-related functional reorganization in non-acute stroke patients following left and right hemisphere subcortical lesions, and its relationship with clinical assessments. METHODS: In this study, we used resting-state fMRI to calculate distance-dependent (i.e., short- and long-range) FCD in 25 left subcortical stroke (LSS) patients, 22 right subcortical stroke (RSS) patients, and 39 well-matched healthy controls (HCs). Then, we compared FCD differences among the three groups and assessed the correlation between FCD alterations and paralyzed motor function using linear regression analysis. RESULTS: Our findings demonstrated that the left inferior frontal gyrus displayed distance-independent FCD changes, while the bilateral supplementary motor area, cerebellum, and left middle occipital gyrus exhibited distance-dependent FCD alterations in two patient subgroups compared with HCs. Furthermore, we observed a positive correlation between increased FCD in the bilateral supplementary motor area and the motor function of lower limbs, and a negative correlation between increased FCD in the left inferior frontal gyrus and the motor function of both upper and lower limbs across all stroke patients. These associations were validated by using a longitudinal dataset. CONCLUSIONS: The FCD in the cerebral and cerebellar cortices shows distance-related changes in non-acute stroke patients with motor dysfunction, which may serve as potential biomarkers for predicting motor outcomes after stroke. These findings enhance our comprehension of the neurobiological mechanisms driving non-acute stroke. TRIAL REGISTRATION: All data used in the present study were obtained from a research trial registered with the ClinicalTrials.gov database (NCT05648552, registered 05 December 2022, starting from 01 January 2022).