Validation of Simplified Visual Acuity Testing Protocols in Amyotrophic Lateral Sclerosis

Source: Neuro-Ophthalmology - Category: Opthalmology Authors: Source Type: research

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ConclusionWhile our sample size limits statistical confidence about the differences observed in this study, it was possible to measure and quantify inter-individual and cohort variability in a non-invasive manner. Our study also shows the potential for MRI based measurements of CSF geometry and flow to provide information about   the hydrodynamic environment of the spinal subarachnoid space. These dynamics may be studied further to understand the behavior of CSF solute transport in healthy and diseased states.
Source: Fluids and Barriers of the CNS - Category: Neuroscience Source Type: research
Abnormal accumulation of TAR DNA-binding protein 43 (TDP-43), a DNA/RNA binding protein, is a pathological signature of amyotrophic lateral sclerosis (ALS). Missense mutations in the TARDBP gene are also found in...
Source: Molecular Brain - Category: Neuroscience Authors: Tags: Micro report Source Type: research
This study provides strong evidence that following a healthy lifestyle can substantially extend the years a person lives disease-free." Commentary on Recent Evidence for Cognitive Decline to Precede Amyloid Aggregation in Alzheimer's Disease I can't say that I think the data presented in the research noted here merits quite the degree of the attention that it has been given in the popular science press. It is interesting, but not compelling if its role...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Authors: Mahoney CJ, Kiernan MC PMID: 31944304 [PubMed - as supplied by publisher]
Source: Medical Journal of Australia - Category: General Medicine Tags: Med J Aust Source Type: research
Mutations in CAPN1 have recently been reported to cause the spastic paraplegia 76 (SPG76) subtype of hereditary spastic paraplegia (HSP). To investigate the role of CAPN1 in spastic paraplegia and other neurodegenerative diseases, including spinocerebellar ataxia (SCA), early-onset Parkinson's disease (EOPD), and amyotrophic lateral sclerosis (ALS) we conducted a mutation analysis of CAPN1 in a cohort of Chinese patients with SPG, SCA, EOPD, and ALS.
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Source Type: research
Publication date: Available online 16 January 2020Source: Stem Cell ResearchAuthor(s): Rachelle Balez, Tracey Berg, Monique Bax, Sonia Sanz Muñoz, Mauricio C. Cabral-da-Silva, Martin Engel, Dzung Do-Ha, Claire H. Stevens, Dominic Rowe, Shu Yang, Ian P. Blair, Lezanne OoiAbstractDermal fibroblasts were donated by a 43 year old male patient with clinically diagnosed familial amyotrophic lateral sclerosis (ALS), carrying the SOD1E101G mutation. The induced pluripotent stem cell (iPSC) line UOWi007-A was generated using repeated mRNA transfections for pluripotency transcription factors Oct4, Klf4, Sox2, c-Myc, Lin28 and...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
ConclusionsBulbar onset was a significant risk predictor for survival. Slower disease progression correlated with better outcomes. Age of onset may differ among ethnic groups. Male patients are more likely to develop Amyotrophic Lateral Sclerosis (ALS) and have shorter survival duration.
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
The materials here report on efforts to screen for small molecule compounds that can reduce the age-related decline of mitochondrial function observed in neurons - and indeed throughout the body. Screening the contents of compound libraries is a process that might sound simple, and conceptually it is, but it is a complex task to build a cost-effective system and supporting logistics to screen for a novel outcome. In this case the outcome is a reversal of at least some degree of reduced mitochondrial function in neurons from old tissue, as well as improvement in important aspects of neural function. Every cell contai...
Source: Fight Aging! - Category: Research Authors: Tags: Medicine, Biotech, Research Source Type: blogs
Authors: Kassubek J, Müller HP Abstract Introduction: In the last decade, multiparametric magnetic resonance imaging (MRI) has achieved tremendous advances in applications to amyotrophic lateral sclerosis (ALS) to increase the understanding of the associated pathophysiology. The aim of this review is to summarize recent progress in the development of MRI-based techniques aiming to support the clinical diagnosis in ALS.Areas covered: The review of structural and functional MRI applications to ALS and its variants (restricted phenotypes) is focused on the potential of MRI techniques which contribute to the diagnost...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research
Gitika Batra, Manav Jain, Rahul Soloman Singh, Amit Raj Sharma, Ashutosh Singh, Ajay Prakash, Bikash MedhiIndian Journal of Pharmacology 2019 51(6):418-425 Amyotrophic lateral sclerosis (ALS) is an untreatable and fatal neurodegenerative disease that is identified by the loss of motor neurons in the spinal cord, brain stem, and motor cortex which theatrically reduces life expectancy. Although the primary cause of ALS remains unclear, its heterogeneity put forward for consideration of association with various factors, including endogenous and/or environmental ones, which may be involved in progressive motor neuron stress t...
Source: Indian Journal of Pharmacology - Category: Drugs & Pharmacology Authors: Source Type: research
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