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CSF neurofilament proteins as diagnostic and prognostic biomarkers for amyotrophic lateral sclerosis

AbstractElevated cerebrospinal fluid (CSF), Neurofilament Light (NF-L) and phosphorylated Heavy (pNF-H) chain levels have been found in Amyotrophic Lateral Sclerosis (ALS), with studies reporting a correlation of both neurofilaments (NFs) with the disease progression. Here, we measured NF-L and pNF-H concentrations in the CSF of ALS patients from a single tertiary Center and investigated their relationship with disease-related variables. A total of 190 ALS patients (Bulbar, 29.9%; Spinal, 70.1%;M/F = 1.53) and 130 controls with mixed neurological diseases were recruited. Demographic and clinical variables were recorded, and ΔFS was used to rate the disease progression. Controls were divided into two cohorts: (1) patients with non-inflammatory neurological diseases (CTL-1); (2) patients wit h acute/subacute inflammatory diseases and tumors, expected to lead to significant axonal and tissue damage (CTL-2). For each patient and control, CSF was taken at the time of the diagnostic work-up and stored following the published guidelines. CSF NF-L and pNF-H were assayed with commercially avai lable ELISA-based methods. Standard curves (from independent ELISA kits) were highly reproducible for both NFs, with a coefficient of variation 
Source: Journal of Neurology - Category: Neurology Source Type: research

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Source: Journal of Neurochemistry - Category: Neuroscience Authors: Tags: Review Source Type: research
We performed a meta‐analysis to examine associations of occupational exposure to extremely‐low frequency magnetic fields (ELF‐MF) with amyotrophic lateral sclerosis (ALS). Epidemiologic studies were identified in EMBASE and MEDLINE, in reference lists and a specialist database. We included studies that reported risk estimates of ALS in association with occupational ELF‐MF exposure. Summary relative risks (RR) or odds ratios (OR) were obtained with random effect meta‐analysis, and analyses were stratified by type of exposure assessment. This was done to evaluate whether observed heterogeneity between studies could...
Source: Bioelectromagnetics - Category: Radiology Authors: Tags: Research Article Source Type: research
Pathophysiological descriptions of amyotrophic lateral sclerosis (ALS) have broadened in recent years to include recognition of widespread involvement of brain pathways in the ‘connectome’, including corpus callosum, with degenerative neuronal pathology in associated cortical areas, basal ganglia, brainstem, cerebellum and spinal cord. In general, these pathological changes are in the anterior brain and its connections, and thus in the classical motor and also in the emotionally expressive brain, including frontal lobes anterior to primary motor areas and temporal lobes.
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Tags: Editorial Source Type: research
Motor neuron disease in 2017: Progress towards therapy in motor neuron disease, Published online: 19 January 2018; doi:10.1038/nrneurol.2017.186In 2017, dramatic advances have been made in the treatment of motor neuron diseases. New therapies have been approved for spinal muscular atrophy and amyotrophic lateral sclerosis, and a host of other therapies that are currently under development are showing promising results.
Source: Nature Reviews Neurology - Category: Neurology Authors: Source Type: research
The objective of present study was to assess the safety and efficacy of nanocurcumin as an anti-inflammatory and antioxidant agent in adults with amyotrophic lateral sclerosis (ALS). We conducted a 12-month, double-blind, randomized, placebo-controlled trial at a neurological referral center in Iran. Eligible patients with a definite or probable ALS diagnosis were randomly assigned to receive either nanocurcumin (80 mg daily) or placebo in a 1:1 ratio. A computerized random number generator was used to prepare the randomization list. All patients and research investigators were blinded to treatment allocation. The primary ...
Source: Neurotherapeutics - Category: Neurology Source Type: research
In this study, we investigated the presence of the G4C2 repeat expansion in 463 Brazilian probands, of whom 404 had ALS/motor neuron disease (ALS/MND) and 67 FTD, and in 63 healthy controls in the southeastern region of Brazil. The highest frequencies of the C9orf72 mutation were in the ALS-FTD group (50% of familial and 17.6% of sporadic cases), although it was also present in 5% of pure ALS/MND patients (11.8% of familial and 3.6% of sporadic cases) and in 7.1% of pure familial FTD.
Source: Neurobiology of Aging - Category: Neuroscience Authors: Tags: Brief communication Source Type: research
Publication date: Available online 18 January 2018 Source:Stem Cell Reports Author(s): Lara Marrone, Ina Poser, Ian Casci, Julia Japtok, Peter Reinhardt, Antje Janosch, Cordula Andree, Hyun O. Lee, Claudia Moebius, Ellen Koerner, Lydia Reinhardt, Maria Elena Cicardi, Karl Hackmann, Barbara Klink, Angelo Poletti, Simon Alberti, Marc Bickle, Andreas Hermann, Udai Pandey, Anthony A. Hyman, Jared L. Sterneckert Perturbations in stress granule (SG) dynamics may be at the core of amyotrophic lateral sclerosis (ALS). Since SGs are membraneless compartments, modeling their dynamics in human motor neurons has been challenging, thu...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
AbstractRecently, mutations inTBK1 (TANK-binding kinase 1) have been reported to be a cause of amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD) spectrum, but the relationship between them remains unclear owing to the small sample size and low mutation rate. Therefore, we performed a two-stage meta-analysis to investigate the frequency ofTBK1 mutations in ALS/FTD patients and the association between the mutations and risk of ALS/FTD spectrum. In the first stage, 12 studies involving 4173 ALS/FTD patients were included. The frequencies of loss of function (LoF) and missense mutations were 1.0% (95% CI 0.6 &nda...
Source: Neurological Sciences - Category: Neurology Source Type: research
Patients with amyotrophic lateral sclerosis (ALS) find value in genetic testing for the disease, whether or not they have a family history of the condition, according to findings from a survey conducted by U.S. researchers.Reuters Health Information
Source: Medscape Medical News Headlines - Category: Consumer Health News Tags: Neurology & Neurosurgery News Source Type: news
Focus on Neuro Diseases We now have the capability to provide cells of the central and peripheral nervous system from donors with Neuro diseases. These include cells from donors with ALS, AD, PD, and Brain Cancer Donors, to name a few.We have provided cells to virtually all the large Pharmas and many small and mid-size Bio-techs. We have worked with Novartis to gain 21-CFR compliance for cells that they are using for their eye diseases drug discovery programs.I am at your “beck and call” should you have interested in exploring specific capabilities further. You can e-mail or call me at 612-801-1007
Source: Neuromics - Category: Neuroscience Tags: 3-D Cell based Assays ALS Alzheimer ' s Disease Amyotrophic Lateral Sclerosis glioblastomas Parkinson Source Type: news
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