Risk factors for social withdrawal in amyotrophic lateral sclerosis/motor neurone disease

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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research

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Conclusion: Overall, and even though we must account for the limitations of the indirect methods and models used for prevalence estimation, we probably have a very high ALS/MND prevalence in Portugal. It would be important to create registries, particularly in rare diseases, for better organization and distribution of healthcare services and resources, particularly at the level of ventilatory support.Neuroepidemiology
Source: Neuroepidemiology - Category: Epidemiology Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron degenerative disease in adults and has also been proven to be a type of conformational disease associated with protein misfolding and dysfunction. To date, more than 150 distinct genes have been found to be associated with ALS, among which Superoxide Dismutase 1 (SOD1) is the first and the most extensively studied gene. It has been well established that SOD1 mutants-mediated toxicity is caused by a gain-of-function rather than the loss of the detoxifying activity of SOD1. Compared with the clear autosomal dominant inheritance of SOD1 mutants in ALS, the po...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
AbstractObjectiveSeveral independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.MethodsComparison of prognostic factors was based on two hospital-based registries. The registry of the German Network for Motor Neuron Diseases contains 3100 patients with ALS. The Chinese registry comprises 2101 patients who were collected between 2003 and 2015 in the metropolitan area of Beij...
Source: Journal of Neurology - Category: Neurology Source Type: research
Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease, is an incurable neurodegenerative condition, characterized by the loss of upper and lower motor neurons. It affects 1-1.8/100,000 individuals worldwide, and the number of cases is projected to increase as the population ages. Thus, there is an urgent need to identify both therapeutic targets and disease-specific biomarkers - biomarkers that would be useful to diagnose and stratify patients into different sub-groups for therapeutic strategies, as well as biomarkers to follow the efficacy of any treatment tested during clinical trials. There is a lack ...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Publication date: Available online 9 May 2019Source: Stem Cell ReportsAuthor(s): Ilary Allodi, Jik Nijssen, Julio Aguila Benitez, Christoph Schweingruber, Andrea Fuchs, Gillian Bonvicini, Ming Cao, Ole Kiehn, Eva HedlundSummaryOculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Toward this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of PHOX2A in neuronal prog...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Proton magnetic resonance spectroscopy (MRS) provides a means of measuring cerebral metabolites in vivo relevant to neurodegeneration. In amyotrophic lateral sclerosis (ALS), neurochemical changes reflecting neuronal loss or dysfunction (decreased N-actylaspartate [NAA]) is most significant in the motor cortex and corticospinal tracts. Other neurochemical changes observed include increased myo-inositol (mIns), a putative marker of gliosis. MRS confirmation of involvement of non-motor regions such as the frontal lobes, thalamus, basal ganglia, and cingulum are consistent with the multi-system facet of motor neuron disease w...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Contributors : Jik Nijssen ; Julio Aguila ; Ilary Allodi ; Eva HedlundSeries Type : Expression profiling by high throughput sequencingOrganism : Mus musculusOculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Towards this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of Phox2a in neuronal progenitors. We demonstrate, using immunocytochemistry and RNA sequencing, tha...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Mus musculus Source Type: research
Contributors : Julio Aguila Benitez ; Jik Nijssen ; Christoph Schweingruber ; Eva HedlundSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensOculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Towards this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of Phox2a in neuronal progenitors. We demonstrate, using electrophysiology, immun...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
This article is protected by copyright. All rights reserved. PMID: 31074054 [PubMed - as supplied by publisher]
Source: The Journal of Physiology - Category: Physiology Authors: Tags: J Physiol Source Type: research
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