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Demyelination diseases

Takumi Kajitani, Toshiki Endo, Tomoo Inoue, Kenichi Sato, Yasushi Matsumoto, Teiji Tominaga
The authors report the case of a 70-year-old woman with lumbar spinal epidural arteriovenous fistula (SEDAVF) who experienced subarachnoid hemorrhage (SAH) after a diagnostic lumbar puncture. According to the literature, perimedullary spinal vein enlargement is a hallmark of spinal vascular diseases; however, there are certain cases in which routine sagittal MRI fails to disclose signal flow voids. In such cases, patients may undergo a lumbar tap to investigate the possible causes of spinal inflammatory or demyelinating disease...
August 10, 2018: Journal of Neurosurgery. Spine
Samila Alves Silva, Patrícia Silva Cunha, Araceli Santos Brito, Rosana Barros Souza, Sandra Lúcia Euzébio Ribeiro
Devic's disease or neuromyelitis optica (NMO) is an autoimmune, inflammatory and demyelinating pathology of the central nervous system that affects the optic nerve and the spinal cord. Diagnosis confirmed by imaging, magnetic resonance (MR) and the presence of the anti-aquaporin 4 antibody (anti-AQP4). We describe two cases of patients with mixed connective tissue disease (MCTD) and Devic's disease, who had anti-AQP4 positive and areas with neuroaxis MR abnormalities, showing this rare association.
April 2018: Acta Reumatológica Portuguesa
G O Omotoso, R M Kolo, T Afolabi, R Jaji-Sulaimon, I T Gbadamosi
Cuprizone-induced neurotoxicity has severally been used to study demyelinating diseases like multiple sclerosis(MS), adversely affecting both the white and grey matters of the brain. Lesions have been observed in different regions ofthe brain including, corpus callosum, neocortex and the hippocampal formation. The current study explored the role ofMoringa oleifera leaf extract in restoring the resultant histomorphological changes in cuprizone-induced hippocampaldamage in Wistar rats. Twenty adult female Wistar rats with average weight of 163...
June 30, 2018: Nigerian Journal of Physiological Sciences: Official Publication of the Physiological Society of Nigeria
H Hajjar, H Boukhaddaoui, A Rizgui, C Sar, J Berthelot, C Perrin-Tricaud, H Rigneault, N Tricaud
Myelin sheath produced by Schwann cells covers and nurtures axons to speed up nerve conduction in peripheral nerves. Demyelinating peripheral neuropathies result from the loss of this myelin sheath and so far, no treatment exists to prevent Schwann cell demyelination. One major hurdle to design a therapy for demyelination is the lack of reliable measures to evaluate the outcome of the treatment on peripheral myelin in patients but also in living animal models. Non-linear microscopy techniques SHG, THG and CARS were used to image myelin ex vivo and in vivo in the sciatic nerve of healthy and demyelinating mice and rats...
August 9, 2018: Journal of Biophotonics
Matthew Cummings, Anitha Christy Sigamani Arumanayagam, Picheng Zhao, Sunil Kannanganat, Olaf Stuve, Nitin J Karandikar, Todd N Eagar
Multiple Sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) where pathology is thought to be regulated by autoreactive T cells of the Th1 and Th17 phenotype. In this study we sought to understand the functions of Presenilin 1 (PSEN1) in regulating T cell effector responses in the experimental autoimmune encephalomyelitis (EAE) murine model of MS. PSEN1 is the catalytic subunit of γ-secretase a multimolecular protease that mediates intramembranous proteolysis. γ-secretase is known to regulate several pathways of immune importance...
2018: PloS One
Barbara Manconi, Barbara Liori, Tiziana Cabras, Federica Vincenzoni, Federica Iavarone, Lorena Lorefice, Eleonora Cocco, Massimo Castagnola, Irene Messana, Alessandra Olianas
Multiple sclerosis is a chronic disease of the central nervous system characterized by inflammation, demyelination and neurodegeneration which is of undetermined origin. To date a single diagnostic test of multiple sclerosis does not exists and novel biomarkers are demanded for a more accurate and early diagnosis. In this study, we performed the quantitative analysis of 119 salivary peptides/proteins from 49 multiple sclerosis patients and 54 healthy controls by a mass spectrometry-based top-down proteomic approach...
August 4, 2018: Journal of Proteomics
Claudia Sommer
PURPOSE OF REVIEW: To give an overview of recent data on the use of nerve and skin biopsy as a diagnostic tool in neuropathies. RECENT FINDINGS: Axonal damage in a biopsy from a patient with chronic inflammatory demyelinating polyradiculoneuropathy may point to the presence of autoantibodies to paranodal proteins. In nonsystemic vasculitis of the peripheral nervous system, nerve biopsy is still the only means to make a definite diagnosis. Increased autophagy has been found in idiopathic neuropathy and may also be a common final pathway in various types of neuropathy...
August 4, 2018: Current Opinion in Neurology
Emmanuel E Comoy, Jacqueline Mikol, Jean-Philippe Deslys
The recently reevaluated high prevalence of healthy carriers (1/2,000 in UK) of variant Creutzfeldt-Jakob Disease (v-CJD), whose blood might be infectious, suggests that the evolution of this prion disease might not be under full control as expected. After experimental transfusion of macaques and conventional mice with blood derived from v-CJD exposed (human and animal) individuals, we confirmed in these both models the transmissibility of v-CJD, but we also observed unexpected neurological syndromes transmissible by transfusion: despite their prion etiology confirmed through transmission experiments, these original cases would escape classical prion diagnosis, notably in the absence of detectable abnormal PrP with current techniques...
August 6, 2018: Prion
Wenting Li, Hao Wu, Chong Gao, Dan Yang, Depo Yang, Jiangang Shen
Multiple sclerosis (MS) is a neuroinflammatory disease in central nervous system (CNS) without effective treatment or medication yet. With high prevalence of MS patients worldwide and poor therapeutic outcome, seeking novel therapeutic strategy for MS is timely important. Radix Rehmanniae (RR), a typical Chinese Medicinal herb, has been used for neuroinflammatory diseases in Traditional Chinese Medicine for centuries. However, scientific evidence and underlying mechanisms of RR for MS are unclear. In this study, we tested the hypothesis that RR could attenuate the progress and severity of MS via suppressing macrophage-derived nitrative damage and inflammation by using experimental autoimmune encephalomyelitis (EAE) model for mimicking MS pathology...
2018: Frontiers in Physiology
Florian Hansmann, Nicole Jungwirth, Ning Zhang, Thomas Skripuletz, Veronika Maria Stein, Andrea Tipold, Martin Stangel, Wolfgang Baumgärtner
In recent years stem cell therapies have been broadly applied in various disease models specifically immune mediated and degenerative diseases. Whether adipose-derived stem cells might represent a useful therapeutic option in virus-triggered central nervous system diseases has not been investigated so far. Theiler's murine encephalomyelitis (TME) and canine distemper encephalitis are established, virus-mediated animal models sharing many similarities with multiple sclerosis (MS). Canine adipose-derived stem cells (ASC) were selected since dogs might serve as an important translational model for further therapeutic applications...
August 2018: Veterinary Immunology and Immunopathology
Isidro Ferrer
Oligodendrocytes are in contact with neurons, wrap axons with a myelin sheath that protects their structural integrity, and facilitate nerve conduction. Oligodendrocytes also form a syncytium with astrocytes which interacts with neurons, promoting reciprocal survival mediated by activity and by molecules involved in energy metabolism and trophism. Therefore, oligodendrocytes are key elements in the normal functioning of the central nervous system. Oligodendrocytes are affected following different insults to the central nervous system including ischemia, traumatism, and inflammation...
August 2, 2018: Progress in Neurobiology
Manuela de Oliveira Fragomeni, Denis Bernardi Bichuetti, Enedina Maria Lobato Oliveira
BACKGROUND: Multiple Sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are acquired demyelinating syndromes of the central nervous system more frequently in young adults and their beginning before 18 years of age is rare. They are autoimmune diseases with distinct pathophysiology, clinical presentation, treatment and prognoses. During childhood these conditions often present similar clinical features and differential diagnosis among pNMOSD, pMS and acute disseminated encephalomyelitis (ADEM) is still difficult at disease onset...
July 20, 2018: Multiple Sclerosis and related Disorders
Christina Mayer, Claus Bruehl, Emma L Salt, Ricarda Diem, Andreas Draguhn, Richard Fairless
Retinal ganglion cells (RGCs), a diverse body of neurons which relay visual signals from the retina to the higher processing regions of the brain, are susceptible to neurodegenerative processes in several diseases affecting the retina. Previous evidence shows that RGCs are damaged at early stages of autoimmune optic neuritis, prior to subsequent degeneration of the optic nerve. In order to study cell type-specific vulnerability of RGCs we performed immunohistochemical and patch clamp electrophysiological analyses of RGCs following induction of autoimmune optic neuritis using the experimental autoimmune encephalomyelitis model in Brown Norway rats...
July 31, 2018: Neuroscience
Andrea Manterola, Ana Bernal-Chico, Raffaela Cipriani, Manuel Canedo-Antelo, Álvaro Moreno-García, Mar Martín-Fontecha, Fernando Pérez-Cerdá, María Victoria Sánchez-Gómez, Silvia Ortega-Gutiérrez, J Mark Brown, Ku-Lung Hsu, Benjamin Cravatt, Carlos Matute, Susana Mato
Multiple sclerosis (MS) is a chronic demyelinating disease of unknown etiology in which tissue pathology suggests both immune-dependent attacks to oligodendroglia and primary oligodendrocyte demise. The endocannabinoid system has been crucially involved in the control of autoimmune demyelination and cannabinoid-based therapies exhibit therapeutic potential, but also limitations, in MS patients. In this context, growing evidence suggests that targeting the hydrolysis of the main endocannabinoid 2-arachidonoylglycerol (2-AG) may offer a more favorable benefit-to-risk balance in MS than existing cannabinoid medicines...
July 31, 2018: Biochemical Pharmacology
R Fledrich, T Abdelaal, L Rasch, V Bansal, V Schütza, B Brügger, C Lüchtenborg, T Prukop, J Stenzel, R U Rahman, D Hermes, D Ewers, W Möbius, T Ruhwedel, I Katona, J Weis, D Klein, R Martini, W Brück, W C Müller, S Bonn, I Bechmann, K A Nave, R M Stassart, M W Sereda
In patients with Charcot-Marie-Tooth disease 1A (CMT1A), peripheral nerves display aberrant myelination during postnatal development, followed by slowly progressive demyelination and axonal loss during adult life. Here, we show that myelinating Schwann cells in a rat model of CMT1A exhibit a developmental defect that includes reduced transcription of genes required for myelin lipid biosynthesis. Consequently, lipid incorporation into myelin is reduced, leading to an overall distorted stoichiometry of myelin proteins and lipids with ultrastructural changes of the myelin sheath...
August 2, 2018: Nature Communications
Ching Soong Khoo, Alyaa Hassan Ali, Rabani Remli, Hui Jan Tan
Guillain-Barré syndrome (GBS) is an acute immune-mediated demyelinating disease. Early recognition of this disease is crucial as it can progress to life-threatening conditions such as respiratory failure or autonomic dysfunction. Typical clinical manifestations of GBS include progressive weakness of the limbs, bulbar, facial muscles and ophthalmoplegia. Sensory level and bladder dysfunction are more suggestive of acute myelopathy. We report a case of GBS presenting with acute urinary retention and T6 sensory level, which was successfully treated with plasma exchange...
August 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
Francisco Javier Domínguez Díez
Treatment with anti-tumor necrosis factor alpha (anti-TNF α) drugs may lead to demyelinating polyneuropathies. Here, we present the case of a patient with rheumatic disease who developed sensory polyneuropathy probably related to anti-TNF α drugs. The patient was diagnosed with undifferentiated arthritis during treatment with weekly injection of golimumab. She presented a progressive picture of paresthesia of all four limbs, with distal and symmetrical predominance, associated with mild dysarthria and universal areflexia, except for the Achilles reflex...
June 22, 2018: European Journal of Rheumatology
Amandine Duchesne, Anne Vaiman, Magali Frah, Sandrine Floriot, Sabrina Legoueix-Rodriguez, Anne Desmazières, Sébastien Fritz, Christian Beauvallet, Olivier Albaric, Eric Venot, Maud Bertaud, Romain Saintilan, Raphaël Guatteo, Diane Esquerré, Julien Branchu, Anaïs Fleming, Alexis Brice, Frédéric Darios, Jean-Luc Vilotte, Giovanni Stevanin, Didier Boichard, Khalid Hamid El Hachimi
Hereditary spastic paraplegias (HSPs) are clinically and genetically heterogeneous human neurodegenerative diseases. Amongst the identified genetic causes, mutations in genes encoding motor proteins such as kinesins have been involved in various HSP clinical isoforms. Mutations in KIF1C are responsible for autosomal recessive spastic paraplegia type 58 (SPG58) and spastic ataxia 2 (SPAX2). Bovines also develop neurodegenerative diseases, some of them having a genetic aetiology. Bovine progressive ataxia was first described in the Charolais breed in the early 1970s in England and further cases in this breed were subsequently reported worldwide...
August 1, 2018: PLoS Genetics
Vrushali Mangale, Laura L McIntyre, Craig M Walsh, Jeanne F Loring, Thomas E Lane
Multiple sclerosis (MS) is a central nervous system (CNS) disease characterized by chronic neuroinflammation, demyelination, and axonal damage. Infiltration of activated lymphocytes and myeloid cells are thought to be primarily responsible for white matter damage and axonopathy. A number of FDA-approved therapies exist that impede activated lymphocytes from entering the CNS thereby limiting new lesion formation in patients with relapse-remitting forms of MS (RRMS). However, a significant challenge within the field of MS research is to develop effective and sustained therapies that allow for axonal protection and remyelination...
August 1, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Qian Li, Abigail Weiland, Xuemei Chen, Xi Lan, Xiaoning Han, Frederick Durham, Xi Liu, Jieru Wan, Wendy C Ziai, Daniel F Hanley, Jian Wang
Although intracerebral hemorrhage (ICH) is a devastating disease worldwide, the pathologic changes in ultrastructure during the acute and chronic phases of ICH are poorly described. In this study, transmission electron microscopy was used to examine the ultrastructure of ICH-induced pathology. ICH was induced in mice by an intrastriatal injection of collagenase. Pathologic changes were observed in the acute (3 days), subacute (6 days), and chronic (28 days) phases. Compared with sham animals, we observed various types of cell death in the injured striatum during the acute phase of ICH, including necrosis, ferroptosis, and autophagy...
2018: Frontiers in Neurology
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