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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A new framework for analysis of three-dimensional shape and architecture of human skeletal muscles from in vivo imaging data.

Cerebral Palsy

A new framework is presented for comprehensive analysis of the three-dimensional shape and architecture of human skeletal muscles from magnetic res...

Recent Investigations on Neurotransmitters' Role in Acute White Matter Injury of Perinatal Glia and Pharmacotherapies-Glia Dynamics in Stem Cell Therapy.

Cerebral Palsy

Periventricular leukomalacia (PVL) and cerebral palsy are two neurological disease conditions developed from the premyelinated white matter ischemi...

Foot orthoses for treating paediatric flat feet.

Cerebral Palsy

Paediatric flat feet are a common presentation in primary care; reported prevalence approximates 15%. A minority of flat feet can hurt and limit gait. There is no optimal strategy, nor consensus, for using foot orthoses (FOs) to treat paediatric flat feet.

To assess the benefits and harms of foot orthoses for treating paediatric flat feet.

We followed standard methods recommended by Cochrane.

We included 16 trials with 1058 children, aged 11 months to 19 years, with flexible flat feet. Distinct flat foot presentations included asymptomatic, juvenile idiopathic arthritis (JIA), symptomatic and developmental co-ordination disorder (DCD). The trial interventions were FOs, footwear, foot and rehabilitative exercises, and neuromuscular electrical stimulation (NMES). Due to heterogeneity, we did not pool the data. Most trials had potential for selection, performance, detection, and selective reporting bias. No trial blinded participants. We present the results separately for asymptomatic (healthy children) and symptomatic (children with JIA) flat feet. The certainty of evidence was very low to low, downgraded for bias, imprecision, and indirectness. Three comparisons were evaluated across trials: CFO versus shoes; PFO versus shoes; CFO versus PFO. Asymptomatic flat feet 1. CFOs versus shoes (1 trial, 106 participants): low-quality evidence showed that CFOs result in little or no difference in the proportion without pain (10-point visual analogue scale (VAS)) at one year (risk ratio (RR) 0.85, 95% confidence interval (CI) 0.67 to 1.07); absolute decrease (11.8%, 95% CI 4.7% fewer to 15.8% more); or on withdrawals due to adverse events (RR 1.05, 95% CI 0.94 to 1.19); absolute effect (3.4% more, 95% CI 4.1% fewer to 13.1% more). 2. PFOs versus shoes (1 trial, 106 participants): low to very-low quality evidence showed that PFOs result in little or no difference in the proportion without pain (10-point VAS) at one year (RR 0.94, 95% CI 0.76 to 1.16); absolute effect (4.7% fewer, 95% CI 18.9% fewer to 12.6% more); or on withdrawals due to adverse events (RR 0.99, 95% CI 0.79 to 1.23). 3. CFOs versus PFOs (1 trial, 108 participants): low-quality evidence found no difference in the proportion without pain at one year (RR 0.93, 95% CI 0.73 to 1.18); absolute effect (7.4% fewer, 95% CI 22.2% fewer to 11.1% more); or on withdrawal due to adverse events (RR 1.00, 95% CI 0.90 to 1.12). Function and quality of life (QoL) were not assessed. Symptomatic (JIA) flat feet 1. CFOs versus shoes (1 trial, 28 participants, 3-month follow-up): very low-quality evidence showed little or no difference in pain (0 to 10 scale, 0 no pain) between groups (MD -1.5, 95% CI -2.78 to -0.22). Low-quality evidence showed improvements in function with CFOs (Foot Function Index - FFI disability, 0 to 100, 0 best function; MD -18.55, 95% CI -34.42 to -2.68), child-rated QoL (PedsQL, 0 to 100, 100 best quality; MD 12.1, 95% CI -1.6 to 25.8) and parent-rated QoL (PedsQL MD 9, 95% CI -4.1 to 22.1) and little or no difference between groups in treatment success (timed walking; MD -1.33 seconds, 95% CI -2.77 to 0.11), or withdrawals due to adverse events (RR 0.58, 95% CI 0.11 to 2.94); absolute difference (9.7% fewer, 20.5 % fewer to 44.8% more). 2. PFOs versus shoes (1 trial, 25 participants, 3-month follow-up): very low-quality evidence showed little or no difference in pain between groups (MD 0.02, 95% CI -1.94 to 1.98). Low-quality evidence showed no difference between groups in function (FFI-disability MD -4.17, 95% CI -24.4 to 16.06), child-rated QoL (PedsQL MD -3.84, 95% CI -19 to 11.33), or parent-rated QoL (PedsQL MD -0.64, 95% CI -13.22 to 11.94). 3. CFOs versus PFsO (2 trials, 87 participants): low-quality evidence showed little or no difference between groups in pain (0 to scale, 0 no pain) at 3 months (MD -1.48, 95% CI -3.23 to 0.26), function (FFI-disability MD -7.28, 95% CI -15.47 to 0.92), child-rated QoL (PedsQL MD 8.6, 95% CI -3.9 to 21.2), or parent-rated QoL (PedsQL MD 2.9, 95% CI -11 to 16.8).

Low to very low-certainty evidence shows that the effect of CFOs (high cost) or PFOs (low cost) versus shoes, and CFOs versus PFOs on pain, function and HRQoL is uncertain. This is pertinent for clinical practice, given the economic disparity between CFOs and PFOs. FOs may improve pain and function, versus shoes in children with JIA, with minimal delineation between costly CFOs and generic PFOs. This review updates that from 2010, confirming that in the absence of pain, the use of high-cost CFOs for healthy children with flexible flat feet has no supporting evidence, and draws very limited conclusions about FOs for treating paediatric flat feet. The availability of normative and prospective foot development data, dismisses most flat foot concerns, and negates continued attention to this topic. Attention should be re-directed to relevant paediatric foot conditions, which cause pain, limit function, or reduce quality of life. The agenda for researching asymptomatic flat feet in healthy children must be relegated to history, and replaced by a targeted research rationale, addressing children with indisputable foot pathology from discrete diagnoses, namely JIA, cerebral palsy, congenital talipes equino varus, trisomy 21 and Charcot Marie Tooth. Whether research resources should continue to be wasted on studying flat feet in healthy children that do not hurt, is questionable. Future updates of this review will address only relevant paediatric foot conditions.

Fat-rich versus carbohydrate-rich nutrition in ALS: a randomised controlled study.

Neurology, Neurosurgery and Psychiatry

There is growing evidence that the course of amyotrophic lateral sclerosis (ALS) may be influenced beneficially by applying high-caloric food supplements (HCSs). However, it is unknown which composition of nutrients offers optimal tolerability and weight gain.

We conducted a randomised controlled study (Safety and Tolerability of Ultra-high-caloric Food Supplements in Amyotrophic Lateral Sclerosis (ALS); TOLCAL-ALS study) in 64 patients with possible, probable or definite ALS according to El Escorial criteria. Patients were randomised into four groups: a high-caloric fatty supplement (HCFS; 405 kcal/day, 100% fat), an ultra-high-caloric fatty supplement (UHCFS; 810 kcal/day, 100% fat), an ultra-high-caloric, carbohydrate-rich supplement (UHCCS; 900 kcal/day, 49% carbohydrates) and an open control (OC) group without any supplement. The primary endpoint was tolerability. Patients were followed up over 4 weeks.

Gastrointestinal side effects were most frequent in the UHCFS group (75.0%), while loss of appetite was most frequent in the UHCCS group (35.3%). During intervention, patients gained +0.9 kg/month of body weight (IQR -0.9 to 1.5; p=0.03) in the HCFS group and +0.9 kg/month (IQR -0.8 to 2.0; p=0.05) in the UHCFS group. A non-significant trend for weight gain (+0.6 kg/month (IQR -0.3 to 1.9; p=0.08)) was observed in the UHCCS group. Patients in OC group continued to lose body weight (-0.5 kg/month, IQR -1.4 to 1.3; p=0.42).

The findings suggest that HCSs frequently cause mild to moderate tolerability issues in patients with ALS, most notably gastrointestinal symptoms in high-fat supplements, and loss of appetite in high-carbohydrate supplements. All three HCSs tested are suited to increase body weight.

Development of novel clinical examination scales for the measurement of disease severity in Creutzfeldt-Jakob disease.

Neurology, Neurosurgery and Psychiatry

To use a robust statistical methodology to develop and validate clinical rating scales quantifying longitudinal motor and cognitive dysfunction in sporadic Creutzfeldt-Jakob disease (sCJD) at the bedside.

Rasch analysis was used to iteratively construct interval scales measuring composite cognitive and motor dysfunction from pooled bedside neurocognitive examinations collected as part of the prospective National Prion Monitoring Cohort study, October 2008-December 2016.A longitudinal clinical examination dataset constructed from 528 patients with sCJD, comprising 1030 Motor Scale and 757 Cognitive Scale scores over 130 patient-years of study, was used to demonstrate scale utility.

The Rasch-derived Motor Scale consists of 8 items, including assessments reliant on pyramidal, extrapyramidal and cerebellar systems. The Cognitive Scale comprises 6 items, and includes measures of executive function, language, visual perception and memory. Both scales are unidimensional, perform independently of age or gender and have excellent inter-rater reliability. They can be completed in minutes at the bedside, as part of a normal neurocognitive examination. A composite Examination Scale can be derived by averaging both scores. Several scale uses, in measuring longitudinal change, prognosis and phenotypic heterogeneity are illustrated.

These two novel sCJD Motor and Cognitive Scales and the composite Examination Scale should prove useful to objectively measure phenotypic and clinical change in future clinical trials and for patient stratification. This statistical approach can help to overcome obstacles to assessing clinical change in rapidly progressive, multisystem conditions with limited longitudinal follow-up.

The unstable hip in children with cerebral palsy: does an acetabuloplasty add midterm stability?

Cerebral Palsy

This study addresses whether an additional pelvic procedure is superior to a varus derotation osteotomy femur (VDRO) alone in unstable hips in children with cerebral palsy (CP).

All patients had unstable hips utilising the Melbourne Cerebral Palsy Hip Classification System (MCPHCS). We compared one group that underwent VDRO alone with one that had a combination of VDRO and Dega osteotomy (VDRO+). Measurements were taken before surgery, postoperatively, two years after surgery and at latest follow-up. Generalised estimating equations were used to account for known and unknown correlations between hips from bilateral cases.

In total, 74 hips in 57 children fulfilled the inclusion criteria. There was no outcome difference between Gross Motor Function Classification System levels III, IV and V. Age at time of operation ranged from three to 16 years (mean 9.8 years). Mean follow-up was 49.1 months. In the VDRO group (28 hips) migration percentage (MP) changed from 61% preoperative to a final value of 35.7%. In the VDRO+ group (46 hips) the MP changed from 64.4% to 19.3%. At final follow-up 15 hips (54%) were stable in the VDRO group, 37 hips (83%) in the VDRO+ group. The odds ratio (OR) of hip stability at final follow-up was 3.5-times higher in the VDRO+ group versus the VDRO group (OR = 3.9; 95% confidence interval = 1.5 to 9.7; p = 0.004).

Reconstruction of unstable hips via VDRO + Dega in children with CP provides a higher likelihood of long-term stability than an isolated VDRO.

Level III, retrospective comparative study.

Vision Abnormalities in Children and Young Adults With Cerebral Palsy; A Systematic Review.

Cerebral Palsy

The current study was designed to provide detailed information on the prevalence of ocular abnormalities in patients with cerebral palsy (CP).

Four international online scientific databases, including Web of Science, PubMed, Scopus, and Google Scholar were systemically searched. First, the titles of the articles were evaluated, and if relevant, their abstracts and full texts were reviewed. The quality of the studies was assessed using the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist.

A total of 147 articles were found in the initial search. After applying the exclusion criteria, 65 articles were chosen for further review, from which 17 articles, comprising a total of 1734 patients with CP ranging in age from birth to 22 years, passed the STROBE quality check and were included in this review. The prevalence of ocular abnormalities in the CP patients reported in the evaluated studies ranged between 34% to 100%, with refractive error, strabismus, and nystagmus exhibiting the greatest overall prevalence at 52%, 48%, and 11%, respectively in this population.

Early ocular assessment of children with CP is essential for an accurate diagnosis, personalized rehabilitation and performing early interventions to improve their visual function.

Supporting Ultra Poor People with Rehabilitation and Therapy among families of children with Cerebral Palsy in rural Bangladesh (SUPPORT CP): Protocol of a randomised controlled trial.

Cerebral Palsy

Poverty is a key contributor to delayed diagnosis and limited access to early intervention and rehabilitation for children with cerebral palsy (CP) in rural Bangladesh. 97% of families of children with CP live below the poverty line in Bangladesh. Therefore, in low-and middle-income countries (LMICs), efforts to improve outcomes for children with CP (including health-related quality of life, motor function, communication, and nutritional attainments) should also include measures to improve family economic and social capital. We propose a randomised controlled trial (RCT) to evaluate the effectiveness of an integrated microfinance/livelihood and community-based rehabilitation (IMCBR) program for ultra-poor families of children with CP in rural Bangladesh.

This will be a cluster RCT comparing three arms: (a) integrated microfinance/livelihood and community-based rehabilitation (IMCBR); (b) community-based rehabilitation (CBR) alone; and (c) care-as-usual (i.e. no intervention). Seven clusters will be recruited within each arm. Each cluster will consist of 10 child-caregiver dyads totalling 21 clusters with 210 dyads. Parents recruited in the IMCBR arm will take part in a microfinance/livelihood program and Parent Training Module (PTM), their children with CP will take part in a Goal Directed Training (GDT) program. The programs will be facilitated by specially trained Community Rehabilitation Officers. The CBR arm includes the same PTM and GDT interventions excluding the microfinance/livelihood program. The care-as-usual arm will be provided with information about early intervention and rehabilitation. The assessors will be blinded to group allocation. The duration of the intervention will be 12 months; outcomes will be measured at baseline, 6 months, 12 months, and 18 months.

This will be the first RCT of an integrated microfinance/livelihood and CBR program for children with CP in LMIC settings. Evidence from the study could transform approaches to improving wellbeing of children with CP and their ultra-poor families.

Effectiveness of Modified Sports for Children and Adolescents With Cerebral Palsy: A Pragmatic Study Protocol.

Cerebral Palsy

Sports Stars is a modified sports intervention that aims to prepare individuals with cerebral palsy for the transition from usual physical therapy care to community sports participation. This is a pragmatic randomized controlled trial protocol of a Brazilian applicability of this intervention.

This study will compare the effectiveness of Sports Stars Brazil with standard care. Seventy-six children who walk and adolescents with cerebral palsy will be randomly assigned into an intervention or control group. The intervention group will receive 8 weekly group sessions. Each session will include 1 hour of sports-focused gross motor activity training and teamwork development, centered on popular Brazilian sports. Participation, levels of physical activity and, physical literacy domains will be measured at baseline, postintervention, and at 12-weeks of follow-up.

The investigation of Sports Stars Brazil will provide high-quality evidence regarding the effectiveness of modified sports intervention to support participation in sports in this population.

Neuroradiological Mimics of Periventricular Leukomalacia.

Cerebral Palsy

Periventricular leukomalacia (PVL) is a term reserved to describe white matter injury in the premature brain. In this review article, the authors highlight the common and rare pathologies mimicking the chronic stage of PVL and propose practical clinico-radiological criteria that would aid in diagnosis and management.

The authors first describe the typical brain MRI (magnetic resonance imaging) features of PVL. Based on their clinical presentation, pathologic entities and their neuroimaging findings were clustered into distinct categories. Three clinical subgroups were identified: healthy children, children with stable/nonprogressive neurological disorder, and those with progressive neurological disorder. The neuroradiological discriminators are described in each subgroup with relevant differential diagnoses. The mimics were broadly classified into normal variants, acquired, and inherited disorders.

The term "PVL" should be used appropriately as it reflects its pathomechanism. The phrase "white matter injury of prematurity" or "brain injury of prematurity" is more specific. Discrepancies in imaging and clinical presentation must be tread with caution and warrant further investigations to exclude other possibilities.

Can adults with cerebral palsy perform and benefit from ballistic strength training to improve walking outcomes? A mixed methods feasibility study.

Cerebral Palsy

Power bursts of hips and ankle plantar flexors are prerequisites to walking propulsion. However, these power bursts are reduced during gait for persons with cerebral palsy (CP) and mainly in the ankle plantar flexors. Hence, task specific training, such as ballistic strength training, is suggested to increase muscle power in walking but not investigated in adults with CP. Therefore, the aim was to investigate if adults with CP could perform and benefit from ballistic strength training to improve walking, evaluated through physical measures and self-reported measures and interviews.

In this mixed methods feasibility study, eight ambulatory adults (aged 24-56) with spastic CP conducted ballistic strength training on a glideboard targeting the ankle plantarflexors two times a week for eight weeks. The feasibility of the training was assessed through objectives described by Orsmond and Cohn. Before and after the intervention, physical measures (6-Minute Walk Test and the eight-item High-level Mobility Assessment Tool) and self-reported measures (Patient Global Impression of Change, Numeric Pain Rating Scale, Fatigue Impact and Severity Self-Assessment, and Walk-12) were collected. After the intervention, semi-structured interviews explored experiences of this training.

The participants experienced training the ankle plantar flexor as relevant but reported it took about four weeks to coordinate the exercises successfully. Although we observed no changes in the physical performance measures, most participants reported improvements; some felt steadier when standing, walking, and hopping.

This study demonstrated that ballistic strength training was feasible and suitable in adults with CP. However, guidance and a long (4 weeks) familiarization time were reported necessary to master the exercises. Most participants reported self-experienced improvements, although no physical performance measures improved. Thus, prolonged intervention may be required for perceived physical improvements to emerge. Also, other outcome measures sensitive to power output remains to be investigated.

Effect of Treatment of Clinical Seizures vs Electrographic Seizures in Full-Term and Near-Term Neonates: A Randomized Clinical Trial.

Cerebral Palsy

Seizures in the neonatal period are associated with increased mortality and morbidity. Bedside amplitude-integrated electroencephalography (aEEG) has facilitated the detection of electrographic seizures; however, whether these seizures should be treated remains uncertain.

To determine if the active management of electrographic and clinical seizures in encephalopathic term or near-term neonates improves survival free of severe disability at 2 years of age compared with only treating clinically detected seizures.

This randomized clinical trial was conducted in tertiary newborn intensive care units recruited from 2012 to 2016 and followed up until 2 years of age. Participants included neonates with encephalopathy at 35 weeks' gestation or more and younger than 48 hours old. Data analysis was completed in April 2021.

Randomization was to an electrographic seizure group (ESG) in which seizures detected on aEEG were treated in addition to clinical seizures or a clinical seizure group (CSG) in which only seizures detected clinically were treated.

Primary outcome was death or severe disability at 2 years, defined as scores in any developmental domain more than 2 SD below the Australian mean assessed with Bayley Scales of Neonate and Toddler Development, 3rd ed (BSID-III), or the presence of cerebral palsy, blindness, or deafness. Secondary outcomes included magnetic resonance imaging brain injury score at 5 to 14 days, time to full suck feeds, and individual domain scores on BSID-III at 2 years.

Of 212 randomized neonates, the mean (SD) gestational age was 39.2 (1.7) weeks and 122 (58%) were male; 152 (72%) had moderate to severe hypoxic-ischemic encephalopathy (HIE) and 147 (84%) had electrographic seizures. A total of 86 neonates were included in the ESG group and 86 were included in the CSG group. Ten of 86 (9%) neonates in the ESG and 4 of 86 (4%) in the CSG died before the 2-year assessment. The odds of the primary outcome were not significantly different in the ESG group compared with the CSG group (ESG, 38 of 86 [44%] vs CSG, 27 of 86 [31%]; odds ratio [OR], 1.83; 95% CI, 0.96 to 3.49; P = .14). There was also no significant difference in those with HIE (OR, 1.77; 95% CI, 0.84 to 3.73; P = .26). There was evidence that cognitive outcomes were worse in the ESG (mean [SD] scores, ESG: 97.4 [17.7] vs CSG: 103.8 [17.3]; mean difference, -6.5 [95% CI, -1.2 to -11.8]; P = .01). There was little evidence of a difference in secondary outcomes, including time to suck feeds, seizure burden, or brain injury score.

Treating electrographic and clinical seizures with currently used anticonvulsants did not significantly reduce the rate of death or disability at 2 years in a heterogeneous group of neonates with seizures.

http://anzctr.org.au Identifier: ACTRN12611000327987.