To identify a novel physiologic marker of bulbar motor involvement in amyotrophic lateral sclerosis (ALS) based on jaw muscle synergies.

Muscle synergies were extracted from the surface electromyographic recordings of five jaw muscles during speech in 11 individuals at the prodromal or symptomatic stages of bulbar ALS and 10 neurologically-healthy controls, using non-negative matrix factorization. The disrupted muscle synergies in ALS were identified; their efficacy in differentiating individuals with ALS from healthy controls and relation to the previously-reported kinematic and functional speech deficits were determined.

An antagonist synergy (i.e., masseter×digastric) was significantly disrupted in ALS, which differentiated individuals with ALS from healthy controls with 82% sensitivity and 90% specificity. Such a disruption occurred prodromally and was associated with slowed jaw movement and reduced speaking rate across the range of severity.

The disruption of jaw antagonist synergy in ALS likely reflects the impact of impaired neural drive on the coordinative functioning of bulbar muscles, which may be used to assess bulbar motor involvement.

Jaw antagonist synergy shows promise as a novel physiologic marker of bulbar motor involvement in ALS, which has the potential to serve as a quantitative measurement tool for bulbar assessment.

Jaw antagonist synergy shows promise as a novel physiologic marker of bulbar motor involvement in ALS, which has the potential to serve as a quantitative measurement tool for bulbar assessment.

Refractory chronic cough (RCC) is a debilitating condition for which there are no licensed treatments. Lidocaine is a nonselective inhibitor of voltage-gated sodium channels with potential antitussive effects, but randomized placebo-controlled studies evaluating its efficacy in RCC are lacking.

To investigate the efficacy of nebulized lidocaine and lidocaine throat spray versus matched placebos in RCC.

This was a randomized, double-blind, double-dummy, placebo-controlled, 3-way crossover study, comparing the effect of single doses of nebulized lidocaine with lidocaine delivered by a throat spray and matched placebo. The primary end point was cough frequency over the 10 hours following treatment. Secondary end points were visual analog scale scores for urge-to-cough and cough severity; an exploratory analysis evaluated hourly cough rates up to 5 hours after treatment.

Twenty-six subjects with RCC were recruited (22 females; mean age, 53.5 ± 12.1 years; FEV

%predicted, 105.2±16.8 L; forced vital capacity %predicted, 112.4 ± 18 L). Lidocaine throat spray, but not nebulized lidocaine, significantly reduced 10-hour cough frequency as compared with placebo (throat spray, 22.6 coughs/h; nebulization, 26.9 coughs/h; and placebos, 27.6 coughs/h; P= .04,). Lidocaine throat spray showed the greatest effect on cough compared with placebo in the first hour after administration (31.7 coughs/h vs 74.2 coughs/h; P= .004). Both nebulizer and spray treatments significantly alleviated urge-to-cough and cough severity visual analog scale scores compared with placebo (P < .05). There were no serious adverse events associated with lidocaine therapy.

Lidocaine throat spray was effective in reducing cough frequency in patients with RCC. Voltage-gated sodium channel inhibitors applied to pharynx have potential as therapies for RCC.

Lidocaine throat spray was effective in reducing cough frequency in patients with RCC. Voltage-gated sodium channel inhibitors applied to pharynx have potential as therapies for RCC.

Chronic granulomatous disease (CGD) is an inherited defect in components of the nicotinamide adenine dinucleotide phosphate oxidase complex that results in potential life-threatening infective and noninfective complications. Hemophagocytic lymphohistiocytosis (HLH) is an unusual but important inflammatory complication of CGD. Optimal management strategies have not yet been identified in children with CGD who develop HLH.

To analyze clinical and laboratory features of HLH in CGD from a tertiary-care center in North India.

A retrospective review of medical records of children with CGD diagnosed in the last 20 years was performed. Clinical and laboratory features of children with CGD who developed HLH were analyzed.

Of 80 patients diagnosed with CGD, 5 (6.25%) had evidence of HLH. All 5 were males; 4 had X-linked CGD and 1 had autosomal recessive CGD (NCF2 defect). Two children with CGD had HLH as the predominant presenting manifestation mimicking the clinical presentation of congenital HLH. Infectious triggers identified were bloodstream infections (n= 3) (Candida albicans, Burkholderia cenocepacia, Francisella noatuensis), pneumonia (n= 4), and splenic abscess (n= 1). We document the first human infection with a fish pathogen, F.noatuensis, in a child with X-linked CGD. Although mortality was seen in 3 children who received only intravenous (IV) immunoglobulin therapy, the other 2 who received IV methylprednisolone pulse therapy survived.

HLH can be a presenting manifestation of CGD, and workup for CGD must be considered in children with HLH. Early recognition with optimal management of both infectious trigger and HLH is very important to prevent mortality.

HLH can be a presenting manifestation of CGD, and workup for CGD must be considered in children with HLH. Early recognition with optimal management of both infectious trigger and HLH is very important to prevent mortality.

The consistency of meningioma is a factor that may influence surgical planning and the extent of resection. The aim of our study is to develop a predictive model of tumor consistency using the radiomic features of preoperative magnetic resonance imaging and the tumor elasticity measured by intraoperative ultrasound elastography (IOUS-E) as a reference parameter.

A retrospective analysis was performed on supratentorial meningiomas that were operated on between March 2018 and July 2020. Cases with IOUS-E studies were included. A semiquantitative analysis of elastograms was used to define the meningioma consistency. MRIs were preprocessed before extracting radiomic features. Predictive models were built using a combination of feature selection filters and machine learning algorithms logistic regression, Naive Bayes, k-nearest neighbors, Random Forest, Support Vector Machine, and Neural Network. A stratified 5-fold cross-validation was performed. Then, models were evaluated using the area under the curve and classification accuracy.

Eighteen patients were available for analysis. Meningiomas were classified as hard or soft according to a mean tissue elasticity threshold of 120.Thebest-ranked radiomic features were obtained from T1-weighted post-contrast, apparent diffusion coefficient map, and T2-weighted images. The combination of Information Gain and ReliefF filters with the Naive Bayes algorithm resulted in an area under the curve of 0.961 and classification accuracy of 94%.

We have developed a high-precision classification model that is capable of predicting consistency of meningiomas based on the radiomic features in preoperative magnetic resonance imaging (T2-weighted, T1-weighted post-contrast, and apparent diffusion coefficient map).

We have developed a high-precision classification model that is capable of predicting consistency of meningiomas based on the radiomic features in preoperative magnetic resonance imaging (T2-weighted, T1-weighted post-contrast, and apparent diffusion coefficient map).

The minimum clinically important difference (MCID), an important concept to evaluate the effectiveness of treatments, might not be a single „magical” constant for any given health-related quality of life (HRQoL) scale. Thus, we analyzed the effects of various factors on MCIDs for several HRQoL measures in an adult spinal deformity population.

Surgical and nonsurgical patients from a multicenter adult spinal deformity database who had completed pretreatment and 1-year follow-up questionnaires (Core Outcome Measures Index [COMI], Oswestry Disability Index [ODI], Medical Outcomes Study 36-item short-form questionnaire, 22-item Scoliosis Research Society Outcomes questionnaire, and an anchor question of „back health”-related change during the previous year) were evaluated. The MCIDs for each HRQoL measure were calculated using an anchor-based method and latent class analysis for the overall population and subpopulations stratified by age, gender, and baseline scores (ODI and COMI) separately for patients with positive versus negative perceptions of change.

Patients with a baseline ODI score of <20, 20-40, and >40 had an MCID of 2.24, 11.35, and 26.57, respectively. Similarly, patients with a baseline COMI score of <2.75, 2.8-5.4, and >5.4 had an MCID of 0.59, 1.38, and 3.67 respectively. The overall MCID thresholds for deterioration and improvement were 0.27 and 2.62 for COMI, 2.23 and 14.31 for ODI, and 0.01 and 0.71 for 22-item Scoliosis Research Society Outcomes questionnaire, respectively.

The results from the present study have demonstrated that MCIDs change in accordance with the baseline scores and direction of change but not by age or gender. The MCID, in its current state, should be considered a concept rather than a constant.

The results from the present study have demonstrated that MCIDs change in accordance with the baseline scores and direction of change but not by age or gender. The MCID, in its current state, should be considered a concept rather than a constant.

Stimulating electrodes for lower extremity motor-evoked potential (LE-MEP) monitoring with transcortical stimulation are usually placed on the medial side of motor cortex convexity, which is not lower extremity but lumbar motor area. Lumbar MEP may be elicited with lower stimulation intensity than LE-MEP through this location, and it is useful to monitor lower extremity motor function intraoperatively.

Intraoperative lumbar and LE-MEP monitoring with transcortical stimulation during surgery of 12 patients with lesions involving the motor cortex from January 2012 to February 2019 at Shinshu University Hospital were reviewed retrospectively. Stimulations were delivered by a train of 5 pulses of anodal constant current stimulation. Stimulating electrode position was determined by motor cortex mapping. Recording needle electrodes were placed on bilateral lumbar muscles and contralateral leg muscles. The threshold-level stimulation method was used for MEP monitoring. The thresholds, monitoring result, and postoperative motor function of lumbar and lower extremities were compared.

The mean baseline thresholds were 19.9 ± 8.9 mA for lumbar MEP and 26.5 ± 11.5 mA for LE-MEP (P= 0.02). Patterns of intraoperative monitoring changes were the same between lumbar and LE-MEP monitoring.

Lumbar MEP was stimulated with lower stimulation intensity than the LE-MEP with the same intraoperative pattern of waveform changes in 12 patients. Lumbar MEP monitoring may be useful for preserving the corticospinal tract of lower extremities intraoperatively.

Lumbar MEP was stimulated with lower stimulation intensity than the LE-MEP with the same intraoperative pattern of waveform changes in 12 patients. Lumbar MEP monitoring may be useful for preserving the corticospinal tract of lower extremities intraoperatively.