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At the onset of stroke-induced hemiparesis, muscle tissue is normal and motoneurones are not overactive. Muscle contracture and motoneuronal overactivity then develop. Motor command impairments are classically attributed to the neurological lesion, but the role played by muscle changes has not been investigated.

Interaction between muscle and command disorders was explored using quantified clinical methodology-the Five Step Assessment. Six key muscles of each of the lower and upper limbs in adults with chronic poststroke hemiparesis were examined by a single investigator, measuring the angle of arrest with slow muscle stretch (X

) and the maximal active range of motion against the resistance of the tested muscle (X

). The coefficient of shortening C

= (X

-X

)/X

(X

, normally expected amplitude) and of weakness C

= (X

-X

)/X

) were calculated to estimate the muscle and command disorders, respectively. Composite C

(CC

) and C

(CC

) were then derived for each limb by averaging the six cors the lower limb particularly, and, beyond a threshold of severity, may alter descending commands. The latter might occur through chronically increased intramuscular tension, and thereby increased muscle afferent firing and activity-dependent synaptic sensitization at the spinal level.

In chronic hemiparesis, muscle shortening affects the lower limb particularly, and, beyond a threshold of severity, may alter descending commands. The latter might occur through chronically increased intramuscular tension, and thereby increased muscle afferent firing and activity-dependent synaptic sensitization at the spinal level.The Poirier-Bienvenu neurodevelopmental syndrome is an autosomal dominant disorder characterized by intellectual disability and epilepsy. The disease is caused by mutations in the CSNK2B gene, which encodes the beta subunit of casein kinase II, and it has important roles in neuron development and synaptic transmission. In this study, five Chinese patients were diagnosed with Poirier-Bienvenu neurodevelopmental syndrome caused by CSNK2B mutations by whole exome sequencing. We detected four different de novo variants of the CSNK2B gene in these five unrelated Chinese patients two novel mutations, namely, c.100delT (p.Phe34fs*16) and c.158_159insA (p.Asp55fs*4), and two recurrent mutations, namely, c.1A>G (p.Met1?) and c.332 G >C (p.R111P). All five patients showed mild-to-profound intellectual disabilities/or learning disabilities and developmental delays, with or without seizures. Although intellectual disability/developmental delay and epilepsy are the most common manifestations of CSNK2B deficiency, the clinical phenotypes of probands are highly variable, and there is no significant correlation between genotype and phenotype. An abnormal stature may be another common manifestation of CSNK2B deficiency. Here, we report the effects of growth hormone (GH) therapy on the patients' linear height. In conclusion, Poirier-Bienvenu neurodevelopmental syndrome is a highly heterogeneous disease caused by mutations in the CSNK2B gene. The phenotype was highly variable, and no significant correlation of genotype and phenotype was found. Patients with short-stature and CSNK2B deficiency may benefit from GH therapy. The identification and characterization of these novel variants will expand the genotypic and phenotypic spectrum of Poirier-Bienvenu neurodevelopmental syndrome.

The number of intratympanic gentamicin (ITG) injections needed to achieve vertigo control in patients with intractable Ménière's disease (MD) may vary from a single dose to several instillations. Changes in different vestibular test results have been used to define an endpoint of treatment, including the decrease of the vestibulo-ocular reflex (VOR) gain elicited by the head-impulse test.

To assess the accuracy of the VOR gain reduction after horizontal canal stimulation, as measured with the video head-impulse test (vHIT) 1 month after the first intratympanic injection, in predicting the need for one or more instillations to control vertigo spells in the long term.

The VOR gain reduction was calculated in 47 patients submitted to (ITG) therapy 1 month after the first instillation.

Single intratympanic treatment with gentamicin has a 59.6% efficacy in vertigo control in the long term. Hearing change in the immediate period after treatment (1 month) is not significant to pre-treatment result and is simred by the reduction in the gain of the VOR but not useful for prognostic purposes.

Functional and structural alterations in the gray matter have been observed in patients with knee osteoarthritis (KOA). However, little is known about white matter changes in KOA. Here, we evaluated fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) to investigate potential alterations in the white matter of patients with KOA.

A total of 166 patients with KOA, along with 88 age- and sex-matched healthy controls were recruited and underwent brain magnetic resonance imaging (MRI). Diffusion tensor imaging (DTI) data were collected and analyzed using tract-based spatial statistics (TBSS). Statistical significances were determined at

< 0.05 and were corrected by the threshold-free cluster enhancement (TFCE) method. Then, we evaluated potential correlations between FA, MD, AD, RD values and disease duration, Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scores, and visual analog scale (VAS) scores.

FA values for the body of co

Patients with KOA exhibited extensive white matter alterations in sensorimotor and pain-related regions. Longitudinal observation studies on the causation between abnormalities in the white matter tracts and KOA is needed in the future.

To investigate the effect of Fufang Huangqi Decoction on the gut microbiota in patients with class I or II myasthenia gravis (MG) and to explore the correlation between gut microbiota and MG (registration number, ChiCTR2100048367; registration website, http//www.chictr.org.cn/listbycreater.aspx; NCBI SRP338707).

In this study, microbial community composition and diversity analyses were carried out on fecal specimens from MG patients who did not take Fufang Huangqi Decoction (control group,

= 8) and those who took Fufang Huangqi Decoction and achieved remarkable alleviation of symptoms (medication group,

= 8). The abundance, diversity within and between habitats, taxonomic differences and corresponding discrimination markers of gut microbiota in the control group and medicated group were assessed.

Compared with the control group, the medicated group showed a significantly decreased abundance of Bacteroidetes (

< 0.05) and significantly increased abundance of Actinobacteria at the phylum level,tabolic disorders. Fufang Huangqi Decoction regulates the gut microbiota in patients with class I or II MG by reducing the abundance of Blautia and Bacteroides and increasing the abundance of Bifidobacterium and Lactobacillus. The correlation between gut microbiota and MG may be related to cell-mediated immunity.Some surgical failures after temporal lobe epilepsy surgery may be due to the presence of an extratemporal epileptogenic zone. Of particular interest is the medial parietal lobe due to its robust connectivity with mesial temporal structures. Seizures in that area may be clinically silent before propagating to the symptomatogenic temporal lobe. In this paper, we present an overview of the anatomical connectivity, semiology, radiology, electroencephalography, neuropsychology, and outcomes in medial parietal lobe epilepsy. Smoothened Agonist concentration We also present two illustrative cases of seizures originating from the precuneus and the posterior cingulate cortex. We conclude that the medial parietal lobe should be strongly considered for sampling by intracranial electrodes in individuals with nonlesional temporal lobe epilepsy, especially if scrutinizing the presurgical data produces discordant findings.

Charcot-Marie-Tooth type 1A (CMT1A) comprises ~50% of all CMT cases. CMT1A is a slowly progressive motor and sensory neuropathy that leads to significant disability. We aimed to investigate the quality of life (QoL) in Serbian patients with CMT1A and to assess sociodemographic and clinical features associated with their QoL.

Forty-five genetically confirmed patients with CMT1A were included -60% women [age 50.4 ± 12.6 years, disease duration 22 (12.5-31.5) years]. SF-36, Medical Research Council (MRC) Sum Score, CMT Examination Score (CMTES), Overall Neuropathy Limitation Scale (ONLS), Beck Depression Inventory (BDI), and Krupp's Fatigue Severity Scale (FSS) were used in the study.

Regarding SF-36, Mental Health and Social Functioning were the scales with the best achievements, whereas Role Physical was the worst domain. Worse QoL in patients with CMT1A was associated with elder age (rho = -0.34,

< 0.05), longer disease duration (rho = -0.31,

< 0.05), more pronounced muscle weakness measuredot a curable disease, it is of interest to identify factors associated with QoL that are amenable to treatment.

In the pivotal DEFINE and CONFIRM trials for dimethyl fumarate (DMF), patterns of brain volume changes were different, potentially due to low sample sizes and because MRIs were analyzed at two different reading centers. We evaluated effects of DMF on brain volume change in patients with multiple sclerosis (MS) through reanalysis of pooled images from DEFINE/CONFIRM trials in one reading center.

MRIs from DEFINE/CONFIRM at weeks 0, 24, 48, and 96 from patients randomized to twice-daily DMF or placebo (PBO) were reanalyzed at the Cleveland Clinic to measure brain parenchymal fraction (BPF). To account for pseudoatrophy, brain volume estimates were re-baselined to calculate changes for weeks 48-96.

Across studies, 301 and 314 patients receiving DMF and PBO, respectively, had analyzable MRIs. In weeks 0-48, mean ± SE percentage change in BPF was -0.44 ± 0.04 vs. -0.34 ± 0.04% in DMF vs. PBO, respectively, whereas in weeks 48-96, mean ± SE percentage change in BPF was -0.27 ± 0.03 vs. -0.41 ± 0.04% in DMF vs. PBO, respectively. The mixed-effect model for repeated measures showed similar results in weeks 48-96, estimated change (95% confidence interval) in BPF was -0.0021 (-0.0027, -0.0016) for DMF vs. -0.0033 (-0.0039, -0.0028) for PBO (35.9% reduction;

= 0.0025).

The lower rate of whole brain volume loss with DMF in this pooled BPF analysis in the second year vs. PBO is consistent with its effects on relapses, disability, and MRI lesions. Brain volume changes in the first year may be explained by pseudoatrophy effects also described in other MS clinical trials.

The lower rate of whole brain volume loss with DMF in this pooled BPF analysis in the second year vs. PBO is consistent with its effects on relapses, disability, and MRI lesions. Brain volume changes in the first year may be explained by pseudoatrophy effects also described in other MS clinical trials.The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a high transmissible infectious disease that primarily impacts the respiratory system and leads to death as it worsens. Ever since the World Health Organization declared the disease as a global pandemic, the pathophysiology, clinical manifestations, and disease prognosis has been discussed in various literature. In addition to impaired respiratory health, the symptoms also indicated the involvement of the cardiovascular and neurological system after SARS-CoV-2 infection. Despite the pulmonary, cardiovascular, and neurological complications, many reports also revealed the prevalence of vestibulocochlear symptoms like dizziness, vertigo, vestibular neuritis, sudden sensorineural hearing loss, and tinnitus. Though many clinical reports and scientific reviews reported the vestibular and cochlear impairments associated with coronavirus disease 2019 (COVID-19) infection, the underlying pathological mechanisms are still unclear and unexplored. In this review, we discussed the published clinical reports, research articles, and literature reviews related to vestibulocochlear manifestations following SARS-CoV-2 infections.

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