The latest medical research on Neuromuscular Medicine

The research magnet gathers the latest research from around the web, based on your specialty area. Below you will find a sample of some of the most recent articles from reputable medical journals about neuromuscular medicine gathered by our medical AI research bot.

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Contribution of basal ganglia activity to REM sleep disorder in Parkinson's disease.

Neurology, Neurosurgery and Psychiatry

Rapid eye movement (REM) sleep behaviour disorder (RBD) is one of the most common sleep problems and represents a key prodromal marker in Parkinson's disease (PD). It remains unclear whether and how basal ganglia nuclei, structures that are directly involved in the pathology of PD, are implicated in the occurrence of RBD.

Here, in parallel with whole-night video polysomnography, we recorded local field potentials from two major basal ganglia structures, the globus pallidus internus and subthalamic nucleus, in two cohorts of patients with PD who had varied severity of RBD. Basal ganglia oscillatory patterns during RBD and REM sleep without atonia were analysed and compared with another age-matched cohort of patients with dystonia that served as controls.

We found that beta power in both basal ganglia nuclei was specifically elevated during REM sleep without atonia in patients with PD, but not in dystonia. Basal ganglia beta power during REM sleep positively correlated with the extent of atonia loss, with beta elevation preceding the activation of chin electromyogram activities by ~200 ms. The connectivity between basal ganglia beta power and chin muscular activities during REM sleep was significantly correlated with the clinical severity of RBD in PD.

These findings support that basal ganglia activities are associated with if not directly contribute to the occurrence of RBD in PD. Our study expands the understanding of the role basal ganglia played in RBD and may foster improved therapies for RBD by interrupting the basal ganglia-muscular communication during REM sleep in PD.

Nutritional status of children with neurodevelopmental disorders: a cross-sectional study at a tertiary-level hospital in northern Bangladesh.

Cerebral Palsy

Malnutrition in children with neurodevelopmental disorders (NDDs) is a significant global public health issue. Nutritional assessment combined with management or advice are essential to produce optimal outcomes.

The objective of this study was to assess nutritional status and the sociodemographic profile of children with neurodevelopmental disorders in Bangladesh.

A cross-sectional study was conducted from December to April 2020 among the population of children with NDDs who presented to the pediatric department of the TMSS Medical College and Rafatullah Community Hospital in Bogura during this period. Socio-demographic data along with anthropometric measurements of the children were taken. Assessment of nutritional status were made using metrics such as z-scores for weight-for-age (WAZ), height-for-age (HAZ), and body mass index-for-age (BAZ). Descriptive statistics (number and percentage) and analytical statistics (chi-square and logistic regression) were included.

58.6% of children displayed malnutrition, with 47.8% showing undernutrition (WHZ / BAZ - 1 SD-≤-3 SD), and 10.8% overnutrition (BAZ > 2SD). Significant negative associations were found between malnutrition and parental education level, urban residency, and monthly family income. Children diagnosed with cerebral palsy exhibited twice the likelihood to be malnourished (AOR 2.39, 95% CI 0.83-6.87). Furthermore, residing in rural regions was associated with an increased risk of experiencing malnutrition, as indicated by an adjusted odds ratio of 1.60 (95% CI 0.12-3.09).

While the results are cross-sectional, over half of children with NDDs were found to be malnourished, suggesting that children with NDD in Bangladesh are vulnerable to developing any form of malnutrition. Therefore, regular assessments and timely nutritional support may improve their situation.

Pharmacological and neurosurgical management of cerebral palsy and dystonia: Clinical practice guideline update.

Cerebral Palsy

Dystonia, typically characterized by slow repetitive involuntary movements, stiff abnormal postures, and hypertonia, is common among individuals wi...

Partnering Early to Provide for Infants At Risk of Cerebral Palsy (PĒPI ARC): protocol for a feasibility study of a regional hub for early detection of cerebral palsy in Aotearoa New Zealand.

Cerebral Palsy

Cerebral palsy (CP) can now be diagnosed in infants with identified CP risk factors as early as three months of age; however, many barriers prevent equitable access to early detection pathways. The "Partnering Early to Provide for Infants At Risk of Cerebral Palsy" feasibility study (PĒPI ARC) seeks to trial a new approach to decrease inequitable health service in Aotearoa New Zealand for high-risk infants and their families. PĒPI ARC incorporates face-to-face clinics, an in-person and virtual Hub, and the use of telehealth to enable flexible access to CP assessments and support for health professionals in early CP detection.

A non-randomised feasibility study was conducted from a tertiary Neonatal Intensive Care Unit (NICU) in Wellington and included seven regional referral centres, servicing nearly 30% of the total population in New Zealand (NZ). The families of infants with a high risk of neurodevelopmental impairment and health professionals interacting with the Hub were invited to participate. Mixed methods were used to evaluate the (i) equitable implementation of an early detection pathway, (ii) acceptability, (iii) demand among families and health professionals, (iv) efficacy in relation to reducing the age of receipt of CP diagnosis, and (v) the experiences around communication and information sharing.

The NZ Health and Disability Ethics Committee approved this study (HDEC: 2022 FULL 13434). The findings will be disseminated in peer-reviewed journals, in conference presentations, and via professional networks.

Australian New Zealand Clinical Trials Registry: ACTRN12623000600640.

Consensus guidelines for the diagnosis and management of isolated sulfite oxidase deficiency and molybdenum cofactor deficiencies.

Cerebral Palsy

Sulfite intoxication is the hallmark of four ultrarare disorders that are caused by impaired sulfite oxidase activity due to genetic defects in the...

Participation experiences of young people with cerebral palsy in key life situations: A qualitative study.

Cerebral Palsy

To explore the participation experiences of adolescents and young adults with cerebral palsy (CP) in key life situations of young adulthood and investigate the impact of a government-funded, disability insurance scheme on participation and health service access.

We conducted a qualitative descriptive study using semi-structured interviews. Sixteen young people with CP (aged 16-30 years; mean age = 24 years 4 months) participated. Interviews were audio-recorded and transcribed verbatim before thematic analysis.

An overarching theme of 'branching out into adulthood' was identified. Participants described early adulthood as a time of change, choice, and challenge. The sub-themes were: (1) making sense of my CP as an adult; (2) people's attitudes towards disability and the impacts on me; (3) roadblocks and workarounds; and (4) participation at the time of the COVID-19 pandemic. Participants reported complex views on the new disability insurance scheme. While access to services and support increased, participants experienced significant difficulty negotiating appropriate funding, resulting in frustration and reduced confidence in the scheme.

Young people with CP experience complexity as they participate during young adulthood. Alongside exploring how their identity is intertwined with having CP, they face significant barriers to participation when navigating relationships, accessing services, and being involved in the community.

Corticosteroids for the prevention and treatment of bronchopulmonary dysplasia: an overview of systematic reviews.

Cerebral Palsy

Bronchopulmonary dysplasia (BPD) remains an important complication of prematurity. Pulmonary inflammation plays a central role in the pathogenesis of BPD, explaining the rationale for investigating postnatal corticosteroids. Multiple systematic reviews (SRs) have summarised the evidence from numerous randomised controlled trials (RCTs) investigating different aspects of administrating postnatal corticosteroids. Besides beneficial effects on the outcome of death or BPD, potential short- and long-term harms have been reported.

The primary objective of this overview was to summarise and appraise the evidence from SRs regarding the efficacy and safety of postnatal corticosteroids in preterm infants at risk of developing BPD.

We searched the Cochrane Database of Systematic Reviews, MEDLINE, Embase, CINAHL, and Epistemonikos for SRs in April 2023. We included all SRs assessing any form of postnatal corticosteroid administration in preterm populations with the objective of ameliorating pulmonary disease. All regimens and comparisons were included. Two review authors independently checked the eligibility of the SRs comparing corticosteroids with placebo, and corticosteroids with different routes of administration and regimens. The included outcomes, considered key drivers in the decision to administer postnatal corticosteroids, were the composite outcome of death or BPD at 36 weeks' postmenstrual age (PMA), its individual components, long-term neurodevelopmental sequelae, sepsis, and gastrointestinal tract perforation. We independently assessed the methodological quality of the included SRs by using AMSTAR 2 (A Measurement Tool to Assess Systematic Reviews) and ROBIS (Risk Of Bias In Systematic reviews) tools. We assessed the certainty of the evidence using GRADE. We provided a narrative description of the characteristics, methodological quality, and results of the included SRs.

We included nine SRs (seven Cochrane, two non-Cochrane) containing 87 RCTs, 1 follow-up study, and 9419 preterm infants, investigating the effects of postnatal corticosteroids to prevent or treat BPD. The quality of the included SRs according to AMSTAR 2 varied from high to critically low. Risk of bias according to ROBIS was low. The certainty of the evidence according to GRADE ranged from very low to moderate. Early initiated systemic dexamethasone (< seven days after birth) likely has a beneficial effect on death or BPD at 36 weeks' PMA (risk ratio (RR) 0.88, 95% confidence interval (CI) 0.81 to 0.95; number needed to treat for an additional beneficial outcome (NNTB) 16, 95% CI 10 to 41; I2 = 39%; 17 studies; 2791 infants; moderate-certainty evidence) and on BPD at 36 weeks' PMA (RR 0.72, 95% CI 0.63 to 0.82; NNTB 13, 95% CI 9 to 21; I2 = 39%; 17 studies; 2791 infants; moderate-certainty evidence). Early initiated systemic hydrocortisone may also have a beneficial effect on death or BPD at 36 weeks' PMA (RR 0.90, 95% CI 0.82 to 0.99; NNTB 18, 95% CI 9 to 594; I2 = 43%; 9 studies; 1376 infants; low-certainty evidence). However, these benefits are likely accompanied by harmful effects like cerebral palsy or neurosensory disability (dexamethasone) or gastrointestinal perforation (both dexamethasone and hydrocortisone). Late initiated systemic dexamethasone (≥ seven days after birth) may have a beneficial effect on death or BPD at 36 weeks' PMA (RR 0.75, 95% CI 0.67 to 0.84; NNTB 5, 95% CI 4 to 9; I2 = 61%; 12 studies; 553 infants; low-certainty evidence), mostly contributed to by a beneficial effect on BPD at 36 weeks' PMA (RR 0.76, 95% CI 0.66 to 0.87; NNTB 6, 95% CI 4 to 13; I2 = 14%; 12 studies; 553 infants; low-certainty evidence). No harmful side effects were shown in the outcomes chosen as key drivers to the decision to start or withhold late systemic dexamethasone. No effects, either beneficial or harmful, were found in the subgroup meta-analyses of late hydrocortisone studies. Early initiated inhaled corticosteroids probably have a beneficial effect on death and BPD at 36 weeks' PMA (RR 0.86, 95% CI 0.75 to 0.99; NNTB 19, 95% CI not applicable; I2 = 0%; 6 studies; 1285 infants; moderate-certainty evidence), with no apparent adverse effects shown in the SRs. In contrast, late initiated inhaled corticosteroids do not appear to have any benefits or harms. Endotracheal instillation of corticosteroids (budesonide) with surfactant as a carrier likely has a beneficial effect on death or BPD at 36 weeks' PMA (RR 0.60, 95% CI 0.49 to 0.74; NNTB 4, 95% CI 3 to 6; I2 = 0%; 2 studies; 381 infants; moderate-certainty evidence) and on BPD at 36 weeks' PMA. No evidence of harmful effects was found. There was little evidence for effects of different starting doses or timing of systemic corticosteroids on death or BPD at 36 weeks' PMA, but potential adverse effects were observed for some comparisons. Lowering the dose might result in a more unfavourable balance of benefits and harms. Moderately early initiated systemic corticosteroids, compared with early systemic corticosteroids, may result in a higher incidence of BPD at 36 weeks' PMA. Pulse dosing instead of continuous dosing may have a negative effect on death and BPD at 36 weeks' PMA. We found no differences for the comparisons of inhaled versus systemic corticosteroids.

This overview summarises the evidence of nine SRs investigating the effect of postnatal corticosteroids in preterm infants at risk for BPD. Late initiated (≥ seven days after birth) systemic administration of dexamethasone is considered an effective intervention to reduce the risk of BPD in infants with a high risk profile for BPD, based on a favourable balance between benefits and harms. Endotracheal instillation of corticosteroids (budesonide) with surfactant as a carrier is a promising intervention, based on the beneficial effect on desirable outcomes without (so far) negative side effects. Pending results of ongoing large, multicentre RCTs investigating both short- and long-term effects, endotracheal instillation of corticosteroids (budesonide) with surfactant as a carrier is not appropriate for clinical practice at present. Early initiated (< seven days after birth) systemic dexamethasone and hydrocortisone and late initiated (≥ seven days after birth) hydrocortisone are considered ineffective interventions, because of an unfavourable balance between benefits and harms. No conclusions are possible regarding early and late inhaled corticosteroids, as more research is needed.

Antibiotics for the treatment of lower respiratory tract infections in children with neurodisability: Systematic review.

Cerebral Palsy

Determine the optimal antibiotic choice for lower respiratory tract infection (LRTI) in children with neurodisability.

Embase, Ovid Emcare and MEDLINE were searched for studies from inception to January 2023. All studies, except case reports, focusing on the antibiotic treatment of LRTI in children, with neurodisabilities were included. Outcomes included length of stay, intensive care admission and mortality.

Nine studies met the inclusion criteria (5115 patients). All the studies were of low quality. The shortest length of stay was with anaerobic and gram-positive cover. Five studies used anaerobic, gram-positive and gram-negative cover (e.g., amoxicillin-clavulanic acid), which was frequently adequate. In one large study, it was better than gram-positive and gram-negative cover alone (e.g. ceftriaxone). Those unresponsive or more unwell at presentation improved faster on Pseudomonas aeruginosa cover (e.g., piperacillin-tazobactam).

In this context, anaerobic, gram-positive and gram-negative cover is just as effective as P. aeruginosa cover, supporting empiric treatment with amoxicillin-clavulanic acid. If there is a failure to improve, broadening to include P. aeruginosa could be considered. This is consistent with a consensus statement on the treatment of LRTI in children with neurodisability. An accepted definition for what constitutes LRTI in this cohort is required before designing prospective randomised trials.

Does the use of higher versus lower oxygen concentration improve neurodevelopmental outcomes at 18-24 months in very low birthweight infants?

Cerebral Palsy

The trial was registered on January 31, 2019, at ClinicalTrials.gov with the Identifier: NCT03825835.

An international cluster, cross-over randomized trial of initial FiO2 of 0.3 or 0.6 during neonatal resuscitation in preterm infants at birth to increase survival free of major neurodevelopmental outcomes at 18 and 24 months corrected age will be conducted. Preterm infants born between 230/7 and 286/7 weeks' gestation will be eligible. Each participating hospital will be randomized to either an initial FiO2 concentration of either 0.3 or 0.6 to recruit for up to 12 months' and then crossed over to the other concentration for up to 12 months. The intervention will be initial FiO2 of 0.6, and the comparator will be initial FiO2 of 0.3 during respiratory support in the delivery room. The sample size will be 1200 preterm infants. This will yield 80% power, assuming a type 1 error of 5% to detect a 25% reduction in relative risk of the primary outcome from 35 to 26.5%. The primary outcome will be a composite of all-cause mortality or the presence of a major neurodevelopmental outcome between 18 and 24 months corrected age. Secondary outcomes will include the components of the primary outcome (death, cerebral palsy, major developmental delay involving cognition, speech, visual, or hearing impairment) in addition to neonatal morbidities (severe brain injury, bronchopulmonary dysplasia; and severe retinopathy of prematurity).

The use of supplementary oxygen may be crucial but also potentially detrimental to preterm infants at birth. The HiLo trial is powered for the primary outcome and will address gaps in the evidence due to its pragmatic and inclusive design, targeting all extremely preterm infants. Should 60% initial oxygen concertation increase survival free of major neurodevelopmental outcomes at 18-24 months corrected age, without severe adverse effects, this readily available intervention could be introduced immediately into clinical practice.

The critical need to accelerate cerebral palsy research with consumer engagement, global networks, and adaptive designs.

Cerebral Palsy

The prevalence of cerebral palsy (CP) varies globally, with higher rates and burden of disease in low- and middle-income countries. CP is a lifelon...

A systematic review of the efficacy of group social skills interventions on social functioning and social participation in children with acquired brain injury or cerebral palsy.

Cerebral Palsy

The aim of this study was to evaluate the efficacy of Group social skills interventions (GSSIs) versus any comparator on social functioning in children aged 5-12 years with acquired brain injury or cerebral palsy.

GSSIs are an evidence-based approach to foster social skills development in children with autism spectrum disorder. Currently, limited literature exploring GSSIs in children with acquired brain injury and cerebral palsy is available.

MEDLINE, SCOPUS, Embase, CINAHL, Cochrane Library, PsycINFO, clinicaltrials.gov, ICTRP and ProQuest Dissertations and Theses were systematically searched. Study screening, risk-of-bias, Grading of Recommendations Assessment, Development and Evaluation and data extraction were performed in duplicate. Six studies were included in the narrative synthesis (one randomised controlled trial and five nonrandomised studies). Results indicate that GSSIs may increase children's social skills as measured on the Social Skills Rating System and Social Skills Questionnaire. Very low certainty evidence was found for improvements in social functioning and competence.

There is low certainty evidence that participation in GSSI may lead to gains in social functioning for children with acquired brain injury or cerebral palsy. Given the certainty of the evidence, these results must be interpreted with caution. Only one randomised controlled trial of GSSIs for children with acquired brain injury was identified, underscoring the need for additional high-quality studies.

Hospital-based surveillance of children with cerebral palsy in Suriname: The Suriname cerebral palsy register.

Cerebral Palsy

To describe the aetiological risk factors, clinical characteristics, access to rehabilitation, and educational status of children with cerebral palsy (CP) in Suriname.

Hospital-based surveillance of children with CP aged younger than 18 years was conducted at the Academic Hospital Paramaribo, Suriname (known as the Suriname CP Register [SUR-CPR]). Data were collected on sociodemographic characteristics, aetiological risk factors, clinical characteristics, rehabilitation, and educational status. Registry data on aetiological risk factors were compared with available national prevalence rates in Suriname. Descriptive statistics were reported.

Between August 2018 and March 2020, 82 children with CP (mean [SD] age 5 years 10 months [3 years 10 months]) attending the Academic Hospital Paramaribo were registered in the SUR-CPR. The mean (SD) age at diagnosis was 5 years 5 months (4 years 1 month). Spastic CP was predominant in 90.8% of children and 58.8% were classified in Gross Motor Function Classification System levels III to V. Overall, 43.9% had preterm birth compared with 13.9% reported nationally (p < 0.001) and 61.6% had birth-related complications compared with 15% reported nationally (p < 0.001). Additionally, 39.1% had birth asphyxia and 23.2% had early feeding difficulties. Sixty-two percent were admitted to the neonatal intensive care unit, 54.0% of whom required ventilation. Most children (82.5%) had CP acquired pre- or perinatally and 17.5% had CP acquired postneonatally. Seventeen percent had never received any rehabilitation services, and 31.9% of the school-aged children were not enrolled in any education system.

The high burden of known aetiological risk factors, delayed diagnosis, and severe functional impairment among children with CP registered at the Academic Hospital Paramaribo is concerning. Public health interventions targeting early diagnosis and early intervention could improve the functional outcome of children with CP in Suriname.