Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study.

Cerebrospinal Fluid Neurofilaments May Discriminate Upper Motor Neuron Syndromes: A Pilot Study. Neurodegener Dis. 2018 Nov 14;18(5-6):255-261 Authors: Zucchi E, Bedin R, Fasano A, Fini N, Gessani A, Vinceti M, Mandrioli J Abstract BACKGROUND: Patients presenting with upper motor neuron (UMN) signs may widely diverge in prognosis, ranging from amyotrophic lateral sclerosis (ALS) to primary lateral sclerosis (PLS) and hereditary spastic paraplegia (hSP). Neurofilaments are emerging as potential diagnostic and prognostic biomarkers for ALS, but the diagnosis of UMN syndromes still relies mostly on clinical long-term observation and on familiarity or genetic confirmation. OBJECTIVES: To test whether phosphorylated neurofilament heavy chain (pNfH) may discriminate different UMN syndromes at diagnosis and to test their prognostic role among these diseases. METHODS: We measured the cerebrospinal fluid (CSF) and serum pNfH of 30 patients presenting with UMN signs and diagnosed with ALS, hSP, and PLS, plus 9 healthy controls (HC). RESULTS: ALS patients had higher levels of pNfH in CSF and serum compared to HC (p
Source: Neuro-Degenerative Diseases - Category: Neurology Authors: Tags: Neurodegener Dis Source Type: research

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Publication date: Available online 20 February 2020Source: MicronAuthor(s): Jessica R. Morrice, Cheryl Y. Gregory-Evans, Christopher A. ShawAbstractMany different types of pathologies can arise in the central nervous system (CNS), such as neurodegeneration. The incidence of neurodegenerative diseases continues to increase, yet the pathogenesis underlying most neurodegenerative diseases, notably in amyotrophic lateral sclerosis (ALS), remains elusive. Neuronal support cells, or glia, are known to play a crucial role in ALS. Microglia are the resident immune cells of the CNS and also have neurotrophic support functions. Thes...
Source: Micron - Category: Biology Source Type: research
, Stević Z Abstract Radicava™ (Edaravone) was approved the Food and Drug Administration (FDA) as a new treatment for amyotrophic lateral sclerosis (ALS). Edaravone is a synthetic antioxidant that specifically targets oxidative damage interacting with lipid radicals in the cell. ALS is a disease were multiple cell types are involved in the devastating loss of motor neurons. Mutations and biochemical changes in various cell types jointly contribute to motor neuron death, disease onset and disease progression. The overall mechanism of neurodegeneration in ALS is still not completely understood. Dying motor neu...
Source: Current Drug Targets - Category: Drugs & Pharmacology Authors: Tags: Curr Drug Targets Source Type: research
In conclusion, PPIA is a disease modifier with prognostic potential. PBMC proteins indicative of alterations in protein and RNA homeostasis are promising biomarkers of ALS, for diagnosis, prognosis and patient stratification. PMID: 32087285 [PubMed - as supplied by publisher]
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research
AbstractIn amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), spinal and lower brainstem motor neurons degenerate, but some motor neuron subtypes are spared, including oculomotor neurons (OMNs). The mechanisms responsible for this selective degeneration are largely unknown, but the molecular signatures of resistant and vulnerable motor neurons are distinct and offer clues to neuronal resilience and susceptibility. Here, we demonstrate that healthy OMNs preferentially expressSynaptotagmin 13 (SYT13) compared to spinal motor neurons. In end-stage ALS patients,SYT13 is enriched in both OMNs and the remaini...
Source: Acta Neuropathologica - Category: Neurology Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is an upper motor neuron disease with an unknown pathogenesis and no effective treatment. A recent study found that treatment of a mouse model of ALS (TgSOD1 mice) intraperitoneally with the mast-cell blocker disodium chromoglycate (cromolyn) had a small but significant effect on disease onset, improvement of neurologic symptoms, and decrease in the expression of proinflammatory cytokines and chemokines in the spinal cord and plasma of the TgSOD1 mice. Treatment with cromolyn also reduced degranulation of mast cells in the tibialis anterior muscle. There was no effect o...
Source: Clinical Therapeutics - Category: Drugs & Pharmacology Authors: Tags: Clin Ther Source Type: research
CONCLUSION: In proposed Auto Regressive (AR) model has been used for Amyotrophic Lateral Sclerosis (ALS) patient data analysis. The 4th order of Yule Walker auto-regressive model is giving best fitting on this problem. PMID: 32008542 [PubMed - in process]
Source: Current Medical Imaging Reviews - Category: Radiology Tags: Curr Med Imaging Rev Source Type: research
Conclusion: This Phase 2 multi-center, randomized, placebo controlled, double blind clinical trial will provide evidence of efficacy and safety of Tecfidera in sporadic ALS.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Study Protocol Clinical Trial Source Type: research
CONCLUSION: The different expression of miRNAs and HDAC4 in genetic ALS vs. SALS and UMN cases is likely to be correlated to different pathogenic mechanisms.
. PMID: 32000889 [PubMed - as supplied by publisher]
Source: Clinical Neuropathology - Category: Pathology Authors: Tags: Clin Neuropathol Source Type: research
Abstract Amyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of motor neurons. Astrocytes from diverse ALS models induce motor neuron death in co-culture. Enhancing NAD+ availability, or increasing the expression of the NAD+-dependent deacylases SIRT3 and SIRT6, abrogates their neurotoxicity in cell culture models. To determine the effect of increasing NAD+ availability in ALS mouse models we used two strategies, ablation of a NAD+-consuming enzyme (CD38) and supplementation with a bioavailable NAD+ precursor (nicotinamide riboside, NR). Deletion of CD38 had no effect in the survival o...
Source: Experimental Neurology - Category: Neurology Authors: Tags: Exp Neurol Source Type: research
nco M Abstract The pleiotropic peptide insulin-like growth factor 1 (IGF-I) regulates human body homeostasis and cell growth. IGF-I activates two major signaling pathways, namely phosphoinositide-3-kinase (PI3K)/protein kinase B (PKB/Akt) and Ras/extracellular signal-regulated kinase (ERK), which contribute to brain development, metabolism and function as well as to neuronal maintenance and survival. In this review, we discuss the general and tissue-specific effects of the IGF-I pathways. In addition, we present a comprehensive overview examining the role of IGF-I in neurodegenerative diseases, such as spinal and ...
Source: Frontiers in Neuroendocrinology - Category: Endocrinology Authors: Tags: Front Neuroendocrinol Source Type: research
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