We report here our studies on IgM reactivity towards peripheral nervous system gangliosides, in motor-neuron diseases (MND) without IgM gammopathies, and in peripheral neuropathies with IgM gammopathies. We showed by enzyme linked immunosorbent assay technique, that anti-GM1 IgM antibodies were often present at a low level in normal controls in contrast to anti-GD1b antibodies, which were never detected in control sera. We evidenced that several steps of the ELISA technique were critical such as the nonaddition of detergent in buffer solutions used for dilutions and for washing and the choice of the ELISA plates. We studied 50 cases of motor-neuron diseases, among which 40 typical cases of Amyotrophic Lateral Sclerosis, only a few had high anti-GM1 antibodies levels, which were always confirmed by immunodetection on thin-layer chromatography. These antibodies were generally directed against the oligosaccharide epitope present also in asialoGM1. No correlation has been as yet established in relation to the clinical state of the patients. In a few cases of polyneuropathies associated with IgM gammopathies, antiganglioside antibodies have been reported. We have found anti-GD1b antibodies to be present in a sensory-motor axonal neuropathy; axonal involvement was evidenced by electrophysiological study.
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http://dx.doi.org/10.1016/0197-0186(92)90049-w | DOI Listing |
J Neurosci
January 2025
Department of Developmental Biology, Washington University School of Medicine, St. Louis, Missouri, 63110, USA.
Neurodegenerative diseases of both the central and peripheral nervous system are characterized by selective neuronal vulnerability, i.e., pathology that affects particular types of neurons.
View Article and Find Full Text PDFBMJ Open
January 2025
Lancashire and South Cumbria MND Care and Research Centre, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK.
Background: Caregivers of people with motor neuron disease (MND) face more negative consequences of caregiving than other terminal illnesses. The impact of this caregiver burden can negatively influence bereavement outcomes.
Objectives: This study aims to explore the support needs of caregivers of people with MND, the types of bereavement services they use, or the reasons for not using bereavement services, and understanding the opportunities and barriers to accessing bereavement services.
Neurology
February 2025
Department of Neurology, University of Michigan, Ann Arbor.
Background And Objectives: An adverse social exposome negatively affects many diseases, but its association with amyotrophic lateral sclerosis (ALS) survival is unknown. This study examined the association between the social exposome measure Area Deprivation Index (ADI) and ALS survival.
Methods: This is a retrospective analysis of patients with ALS at the University of Michigan Pranger ALS Clinic diagnosed after January 1, 2012.
Sci Transl Med
January 2025
University of Strasbourg, INSERM, Strasbourg Translational Neuroscience & Psychiatry STEP-CRBS, UMR-S 1329, 67000 Strasbourg, France.
Sleep alterations have been described in several neurodegenerative diseases yet are currently poorly characterized in amyotrophic lateral sclerosis (ALS). This study investigates sleep macroarchitecture and related hypothalamic signaling disruptions in ALS. Using polysomnography, we found that both patients with ALS as well as asymptomatic and mutation carriers exhibited increased wakefulness and reduced non-rapid eye movement sleep.
View Article and Find Full Text PDFActa Naturae
January 2024
Research Center of neurology, Ministry of Science and Higher Education of the Russian Federation, Moscow, 125367 Russian Federation.
Amyotrophic lateral sclerosis (ALS) is a severe disease of the central nervous system (CNS) characterized by motor neuron damage leading to death from respiratory failure. The neurodegenerative process in ALS is characterized by an accumulation of aberrant proteins (TDP-43, SOD1, etc.) in CNS cells.
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