Advanced searches left 3/3

Amyotrophic Lateral Sclerosis - Europe PMC

Summarized by Plex Scholar
Last Updated: 27 October 2022

* If you want to update the article please login/register

Promising application of a new ulnar nerve compound muscle action potential measurement montage in amyotrophic lateral sclerosis: a prospective cross-sectional study.

In 2020, a new way of measuring ulnar nerve compound muscle activity potential was introduced, adapting the E2 electrode from the abductor digitorum's distal tendon to the middle of the proximal wrist's back. The amplitude of compound muscle action potential-pE2 was significantly lower than that of compound muscle action potential-dE2 sclerosis patients with abductor digiti minimi spontaneous movement and muscular atrophy in amyotrophic lateral sclerosis patients with abductor digiti minimi spontaneous transmission and muscular atrophy. These results indicate that the latest ulnar nerve compound muscle action potential measurement montage reduces the effect of the reference electrode by changing the E2 electrode position, and that this approach is more appropriate for monitoring disease progression than the traditional montage.

Source link: https://europepmc.org/article/MED/36204862


Serum uric acid level predicts the progression of amyotrophic lateral sclerosis following treatment with edaravone.

We investigated whether the treatment effect of edaravone is evident in patients whose uric acid level increased after the treatment with edaravone. After edaravone therapy, Baseline uric acid level and the rate of decline in uric acid were measured. Results After treatment in 26 patients and an increase in 12 patients, the serum uric acid levels decreased. The r/month in patients whose uric acid dropped was much faster than in patients whose uric acid levels increased, but not in patients whose uric acid levels increased. 00394ALSFRSFRS-R/month.

Source link: https://europepmc.org/article/MED/35296219


Genetic analysis of and clinical characteristics associated with ANXA11 variants in a Chinese cohort with amyotrophic lateral sclerosis.

Variants in the annexin A11 gene have been shown to be associated with amyotrophic lateral sclerosis. However, the genetic source and clinical characteristics of ALS patients with ANXA11 variants are largely unknown. On 1587 Chinese patients with ALS, genetic testing was done. We found 20 non-synonymous variants in 29 ALS patients, including one stop-gain, one frameshift, and 18 rare missense variants with seven predicted pathogenic variants in our ALS cohort. Patients with the ANXA11 variants that affect the C-terminal of the protein's C-terminal of the protein's C-terminal had earlier disease development and shorter survival times than those with the N-terminal variant. In addition, Caucasian ANXA11 carriers were more likely to experience cognitive impairment, mainly frontotemporal dementia, than their Asian counterparts. Although more than half of the patients in our study had cognitive impairment, none had FTD. Our study extends the genotypic and phenotypic characteristics of ALS patients with ANXA11 variants.

Source link: https://europepmc.org/article/MED/36280108


MicroRNA signatures in genetic frontotemporal dementia and amyotrophic lateral sclerosis.

We want to investigate all recently reported circulating microRNA signatures as potential biomarkers of genetic FTD and/or ALS by using homogeneous, independent validation cohorts of C9orf72 and GRN mutation carriers. Between 31 controls, 17 C9orf72 diseased people, and 29 C9orf72 patients, we found significant differences in the expression levels of 65 microRNAs from 15 published studies about FTD or ALS. Also compared the expression levels of 30 microRNAs found in five FTD studies among 31 controls, 30 GRN presymptomatic subjects, and 28 GRN patients. Results More than half of the selected microRNAs were differentially expressed in the C9orf72 cohort, although only a small number of microRNAs were differentially expressed in the GRN cohort. Interpretation of FTD and ALS patients using sporadic or mixed cohorts may have the ability to identify microRNAs related to C9orf72-associated disease, but not GRN-associated disease.

Source link: https://europepmc.org/article/MED/36264717


Finding Common Ground on the Site of Onset of Amyotrophic Lateral Sclerosis.

Since its inception as a persistently progressive age with prominent neuromuscular signs that are correlated with upper and lower motor neuron dysfunction, amyotrophic lateral sclerosis has been an enigma since its earliest description as a chronic illness with persistent neuromuscular abnormalities. Although this remains the hallmark of ALS, a significant number of patients will have one or two signs of frontotemporal dysfunction, including a frontotemporal dementia. Both ALS and FTD show an evolution in the metabolism of TAR DNA-binding protein 43, characterized by a shift in nucleocytoplasmic localization within a wide variety of neuronal cytoplasmic inclusions consisting of pathological aggregates of RNA binding proteins. The co-occurrence of one or two network disorders in ALS and FTD is thus a product not of specific neuroanatomic correlates but rather of shared molecular vulnerabilities.

Source link: https://europepmc.org/article/MED/36261296


Impact of the National Amyotrophic Lateral Sclerosis Registry: Analysis of Registry-funded Research.

This paper seeks to investigate the effects of the National Amyotrophic Lateral Sclerosis Registry-funded research efforts in the United States. On study abstraction techniques, key research results and key impact factors were abstracted and reported. Result Since 2012, the National ALS Registry sponsored 21 research studies. With a weighted relative citation ratio of 16. 28, the relative citation ratio averaged 1. 81 with a weighted relative citation ratio of 16. 28. The most common inquiries were environmental and occupational risk factors mainly related to heavy metal exposure and agricultural chemicals, as well as occupations related to those chemicals. Interpretation of the National ALS Registry The National ALS Registry is a multifaceted research platform, one of which is funded research. This Registry-funded study fills a significant gap in the ALS scientific community overall, because it is impossible to prevent and treat a disease without a greater knowledge of its causes.

Source link: https://europepmc.org/article/MED/36259277


Amyotrophic Lateral Sclerosis Clinical Trials and Interpretation of Functional End Points and Fluid Biomarkers: A Review.

Importance: Importance Clinical trials in amyotrophic lateral sclerosis are on the rise; as a result, trial improvements have been introduced to increase effectiveness, outcome assessment have been reassessed, and widespread debate about the amount of data necessary to advance a drug to approval has occurred. Small trials with equivocal medical findings also raise concerns about the evidence that is required to support regulatory approval. While blood neurofilament measurements can predict prognosis in ALS and may be a sensitive measure of biologic function, current findings do not yet support its use as a primary outcome. Although clinical outcomes are the most accurate primary outcome measures, fluid markers indicating biologically important processes will grow in importance as more is discovered about the correlation between such markers and clinical end points.

Source link: https://europepmc.org/article/MED/36251310


Design of Mesoporous Silica Nanoparticles for the Treatment of Amyotrophic Lateral Sclerosis (ALS) with a Therapeutic Cocktail Based on Leptin and Pioglitazone.

Therapeutic agents used to treat ALS are very limited, however combined therapies may be a more effective treatment approach. We've investigated the ability of nanomedicine to create a single platform based on mesoporous silica nanoparticles for the treatment of an ALS animal model with a mixture of chemicals such as leptin and pioglitazone, which have already demonstrated promising therapeutic activity in other neurodegenerative disorders. Body weight loss was investigated, and motor coordination in TDP-43 T315T mice and wild-type mice was tested, and motor coordination was established using the rotarod method. Nonetheless, the disease progression was slower and showed significant improvements in motor results, suggesting that TDP-43 A315T mice treated with MSN-LEP-PIO have less energy consumption in the late stage of ALS symptoms.

Source link: https://europepmc.org/article/MED/36240025


Acid sphingomyelinase inhibition improves motor behavioral deficits and neuronal loss in an amyotrophic lateral sclerosis mouse model.

Amyotrophic lateral sclerosis is an incurable neurodegenerative disorder characterized by the degeneration of motor neurons in the spinal cord. In addition, genetic restriction of ASM enhances motor function impairment and spinal neuronal loss in an ALS mouse model. These results indicate that ASM is a potentially useful target, and that ASM inhibition may be a potential therapeutic strategy for ALS.

Source link: https://europepmc.org/article/MED/36229415


pTDP-43 aggregates accumulate in non-central nervous system tissues prior to symptom onset in amyotrophic lateral sclerosis: a case series linking archival surgical biopsies with clinical phenotypic data.

Our goal was to determine the systemic distribution of pathology in archived non-CNS tissues, obtained as part of routine medical care during life from people with ALS. We examined tissue from 13 people who went on to cause ALS, including sporadic ALS and C9orf72 hexanucleotide repeat expansion. We found pTDP-43 aggregates in various cell types of the GI tract, including macrophages and dendritic cells within the lamina propria, as well as ganglion/neuronal, and glial cells of the myenteric plexus. We should note that in all cases of non-CNS pTDP-43 pathology, aggregates were present prior to ALS diagnosis and in some cases preceded neurological symptom onset by more than ten years. Patients with microscopically unexplained non-CNS symptoms may have occult protein aggregation that may have existed for many years prior to neurological involvement, according to these results.

Source link: https://europepmc.org/article/MED/36226890

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions