Background: Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease of the motor neuron for which no clinically validated biomarkers have been identified.
Methods: We have quantified by ELISA the biomarker phosphoneurofilament heavy chain (pNFH) in the cerebrospinal fluid (CSF) of ALS patients (n=29) and age-matched control patients with other diseases (n=19) by ELISA. Furthermore, we compared protein N-glycosylation of the CSF in ALS patients and controls, by applying a glycomics approach based on liquid chromatography and mass spectrometry.
Results: pNFH levels were significantly higher in ALS patients in comparison with controls (P<0.0001) in particular in fast progressors. The N-glycans found in the CSF were predominantly complex diantennary with sialic acid in α2,3- and α2,6-linkage, and bisecting N-acetylglucosamine-containing structures as well as peripherally fucosylated structures were found. As compared with controls the ALS group had a significant increase of a peak composed of the monosialylated diantennary glycans A2G2S(6)1 and FA2G2S(3)1 (P=0.0348).
Conclusions: Our results underscore the value of pNFH as a biomarker in ALS. In addition, we identified a variation of the N-glycosylation pattern in ALS, suggesting that this change should be explored in future studies as potential biomarker.
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http://dx.doi.org/10.1016/j.cca.2014.09.011 | DOI Listing |
Sci Rep
January 2025
Dept. of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
Primary lateral sclerosis (PLS) is a motor neuron disease (MND) which mainly affects upper motor neurons. Within the MND spectrum, PLS is much more slowly progressive than amyotrophic laterals sclerosis (ALS). `Classical` ALS is characterized by catabolism and abnormal energy metabolism preceding onset of motor symptoms, and previous studies indicated that the disease progression of ALS involves hypothalamic atrophy.
View Article and Find Full Text PDFEur J Neurol
January 2025
Department of Pharmacotherapy, University of Utah Health, Salt Lake City, Utah, USA.
Background: Reduction of intracellular Na accumulation through late Na current inhibition has been recognized as a target for cardiac Ca handling which underlies myocardial contractility and relaxation in heart failure (HF). Riluzole, an Na channel blocker with enhancement of Ca-activated K channel function, used for management of amyotrophic lateral sclerosis (ALS), is effective in suppressing Ca leak and therefore may improve cardiac function.
Objectives: The study aim was to investigate whether riluzole lowers HF incidence.
J Trauma Acute Care Surg
January 2025
From the Department of Surgery (J.H., K.S., G.S.C., C.T., L.R., G.B.); School of Public Health (A.B., O.H., A.S., S.M.); Hennepin Healthcare (S.K.); Department of Emergency Medicine (S.K., M.A.P.); and Hennepin Healthcare, Department of Emergency Medicine (M.A.P.), Minneapolis, Minnesota.
Background: There is conflicting evidence regarding emergency medical service (EMS) provider level of training and outcomes in trauma. We hypothesized that advanced life support (ALS) provider transport is associated with lower mortality compared with basic life support transport.
Methods: We performed secondary analysis of a combined prehospital and in-hospital database of trauma patients utilizing ESO electronic medical records from 2018 to 2022.
Ann Clin Transl Neurol
January 2025
Department of Epidemiology, Columbia University Mailman School of Public Health, New York, USA.
Objective: We examined whether riluzole treatment modifies the associations between the dietary glycemic index (GI) and load (GL) and disease progression in amyotrophic lateral sclerosis (ALS).
Methods: Sporadic ALS patients in the Multicenter Cohort Study of Oxidative Stress who completed a baseline food frequency questionnaire were included (n = 304). Interactions between baseline riluzole treatment and GI/GL on functional decline and tracheostomy-free survival were examined using linear regression and Cox proportional hazard models adjusted for covariates.
Ann Clin Transl Neurol
January 2025
Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
Objective: Patients with amyotrophic lateral sclerosis (ALS) caused by superoxide dismutase 1 (SOD1) gene mutations (SOD1 ALS) treated with tofersen have shown slowing of disease progression, and disease stabilization with recovery of function in some patients. We report our clinical experience with treating patients with SOD1 ALS and the effects of tofersen on outcome measures.
Methods: This was a single-center observational study of patients with SOD1 ALS receiving treatment with tofersen.
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