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Demyelination diseases

Abin Sajan, Soombal Zahid, Jordan Stumph, Daniel Griepp, Sami Saba, Nazish Ilyas, Isabel M Mc Farlane
Background: Acute inflammatory demyelinating polyneuropathy (AIDP) is an uncommon form of neuropathy in HIV-infected patients that can cause pain, sensory disturbance, and motor weakness. Case presentation: A 23-year-old African American male with past medical history of Guillain-Barre Syndrome (GBS), Lyme disease, and sexually transmitted infections including syphilis and chlamydia presented with acute back pain radiating to bilateral lower extremities with worsening right foot weakness for four days...
2019: American Journal of Medical Case Reports
Maria Grazia Maggio, Margherita Russo, Marilena Foti Cuzzola, Massimo Destro, Gianluca La Rosa, Francesco Molonia, Placido Bramanti, Giuseppe Lombardo, Rosaria De Luca, Rocco Salvatore Calabrò
Multiple sclerosis (MS) is a demyelinating neurodegenerative disease with lesions involving the central nervous system. Clinical symptoms consist of disturbances in motor activity (e.g., weakness, spasticity, and tremor), sensory functioning (e.g., pain), visual functions (e.g., diplopia and optic neuritis), besides different cognitive (attention deficit and executive dysfunction) and behavioral abnormalities. This review aims to evaluate the role of VR tools in cognitive and motor rehabilitation of MS patients...
March 18, 2019: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Zhen-Hua Zhao, Zhi-Ting Chen, Rui-Ling Zhou, Yin-Zhou Wang
Charcot‑Marie‑Tooth (CMT) disease is a group of motor and sensory neuropathies with a high degree of pathological and genetic heterogenicity. The present study described 2 patients with CMT in a Chinese Han pedigree. The proband exhibited the classic manifestation of CMT with slowly progressing muscular atrophy and weakness. Electrophysiological examination highlighted axonal and demyelinating features. His mother did not have any symptoms, but did exhibit abnormal electrophysiological results. Next‑generation sequencing technology was employed to screen mutations in the genes associated with inherited motor never diseases...
March 19, 2019: Molecular Medicine Reports
Fernando C Ortiz, Chloé Habermacher, Mariana Graciarena, Pierre-Yves Houry, Akiko Nishiyama, Brahim Nait-Oumesmar, Maria Cecilia Angulo
In demyelinating diseases such as Multiple Sclerosis (MS), demyelination of neuronal fibers impairs impulse conduction and causes axon degeneration. While neuronal activity stimulates oligodendrocyte production and myelination in normal conditions, it remains unclear whether the activity of demyelinated axons restores their loss-of-function in a harmful environment. To investigate this question, we established a model to induce a moderate optogenetic stimulation of demyelinated axons in the corpus callosum at the level of the motor cortex in which cortical circuit activation and locomotor effects were reduced in adult freely moving mice...
March 21, 2019: JCI Insight
Tessa Dhaeze, Catherine Lachance, Laurence Tremblay, Camille Grasmuck, Lyne Bourbonnière, Sandra Larouche, Olivia Saint-Laurent, Marc-André Lécuyer, Rose-Marie Rébillard, Stephanie Zandee, Alexandre Prat
TCR1640 mice, which have a T cell receptor (TCR) directed against MOG92-106, spontaneously develop experimental autoimmune encephalomyelitis. Female mice mostly develop a relapsing-remitting (RR) course and have a higher incidence of disease, while males most frequently suffer from progressive disease, reflecting the unresolved clinical sex discrepancies seen in multiple sclerosis. Herein, we performed adoptive transfers of male and female TCR1640 immune cells into WT animals to investigate if disease course is dependent on the sex of the donor immune cells or on the sex of the recipient animal...
March 21, 2019: JCI Insight
Laurine Becquet, Catalina Abad, Mathilde Leclercq, Camille Miel, Laetitia Jean, Gaëtan Riou, Alain Couvineau, Olivier Boyer, Yossan-Var Tan
BACKGROUND: Orexins (hypocretins, Hcrt) A and B are GPCR-binding hypothalamic neuropeptides known to regulate sleep/wake states and feeding behavior. A few studies have shown that orexin A exhibits anti-inflammatory and neuroprotective properties, suggesting that it might provide therapeutic effects in inflammatory and neurodegenerative diseases like multiple sclerosis (MS). In MS, encephalitogenic Th1 and Th17 cells trigger an inflammatory response in the CNS destroying the myelin sheath...
March 20, 2019: Journal of Neuroinflammation
Hans Lassmann
PURPOSE OF REVIEW: Research on multiple sclerosis (MS) pathogenesis and therapy is to a large extent driven by results obtained in experimental autoimmune encephalomyelitis (EAE). This approach provided deep insights into the mechanism of brain inflammation and immune mediated tissue injury and, thus, most of our currently established therapies for MS patients have been developed with profound contributions of experimental autoimmune research. Recent data, which are summarized in this review article, however, show important differences between EAE and MS...
March 19, 2019: Current Opinion in Neurology
Kazuo Fujihara
PURPOSE OF REVIEW: The diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) has been revised in the past 20 years and pathological and therapeutic data have been accumulated. This review provides an overview of evolution and broadening of the concept of NMOSD. RECENT FINDINGS: NMOSD encompassing brain syndrome as well as optic neuritis and acute myelitis is now classified into aquaporine-4 (AQP)-antibody-seropositive and aquaporine-4 (AQP)-antibody-seronegative diseases, detecting more patients earlier than before...
March 19, 2019: Current Opinion in Neurology
Hatice Ayça Ata Korkmaz
Background/aim: In the present study we aimed to investigate whether the earlobe crease (ELC) might provide predictive information about white matter intensities (WMIs) in the brain that reflect brain aging. Materials and methods: A total of 350 individuals examined from January 2016 to July 2016 were screened. Patients with known demyelinating white matter disease, neurodegenerative disorders, cerebrovascular event history, or brain tumors were excluded from the study...
March 20, 2019: Turkish Journal of Medical Sciences
Sarah M Carpanini, Megan Torvell, Bryan Paul Morgan
The complement system plays critical roles in development, homeostasis, and regeneration in the central nervous system (CNS) throughout life; however, complement dysregulation in the CNS can lead to damage and disease. Complement proteins, regulators, and receptors are widely expressed throughout the CNS and, in many cases, are upregulated in disease. Genetic and epidemiological studies, cerebrospinal fluid (CSF) and plasma biomarker measurements and pathological analysis of post-mortem tissues have all implicated complement in multiple CNS diseases including multiple sclerosis (MS), neuromyelitis optica (NMO), neurotrauma, stroke, amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Parkinson's disease (PD), and Huntington's disease (HD)...
2019: Frontiers in Immunology
Alfredo Iardino, Orlando Garner, Saranya Rajasekar, Avinash Alexander, Anuradha Helekar, Grace Shim, Donald Loveman, Ramachandra Chemitiganti, Kalpana Bhairavarasu
BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease that usually presents in pediatric patients, usually following a viral or bacterial infection. The clinical findings in ADEM include acute neurologic decline that typically presents with encephalopathy, with some cases progressing to multiple sclerosis. An atypical case of ADEM is reported that presented in a middle-aged adult. CASE REPORT A 46-year-old Caucasian man, who had recently emigrated to the US from Ukraine, presented with gait abnormalities that began four days after he developed abdominal cramps...
March 19, 2019: American Journal of Case Reports
Carla Francisco, Katherine Meddles, Emmanuelle Waubant
A novel autoantibody, glial fibrillary acidic protein (GFAP)-IgG, has recently been associated with cases of meningoencephalomyelitis. This entity is still being unraveled. Very few pediatric patients have been identified; thus, the clinical, biological and imaging phenotype remains to be defined. Herein we describe the clinical course of a 6-year-old patient initially suspected to have a demyelinating disease but ultimately diagnosed with GFAP-IgG positive autoimmune meningoencephalomyelitis. We also provide a review of the literature regarding this novel entity...
April 2019: Multiple Sclerosis and related Disorders
Paolo Florent Felisaz, Andrea Poli, Raimondo Vitale, Giovanni Vitale, Carlo Asteggiano, Niels Bergsland, Ilaria Callegari, Elisa Vegezzi, Laura Piccolo, Andrea Cortese, Anna Pichiecchio, Stefano Bastianello
OBJECTIVE: In this study we investigated the potential of magnetic resonance (MR) micro-neurography to detect morphological and relaxometric changes in distal tibial nerves in patients affected with chronic inflammatory demyelinating polyneuropathy (CIDP), and their associations with clinical and electrophysiological features. MATERIALS AND METHODS: 10 subjects affected with CIDP and 10 healthy subjects were examined. Multiple MR parameters, including the number of fascicles (N), fascicles diameter (FD), total fascicles area (FA), epineurium area (EA), total nerve area (NA), fascicles to nerve ratio (FNR) and quantitative T2 and proton density (PD) were investigated on high resolution MR images of the distal tibial nerve...
March 11, 2019: Journal of the Neurological Sciences
Zhixiong Liu, Minbiao Yan, Yaoji Liang, Min Liu, Kun Zhang, Dandan Shao, Rencai Jiang, Li Li, Chaomeng Wang, Daniel R Nussenzveig, Kunkun Zhang, Shaoxuan Chen, Chuanqi Zhong, Wei Mo, Beatriz M A Fontoura, Liang Zhang
Nucleoporins (Nups) are involved in neural development, and alterations in Nup genes are linked to human neurological diseases. However, physiological functions of specific Nups and the underlying mechanisms involved in these processes remain elusive. Here, we show that tissue-specific depletion of the nucleoporin Seh1 causes dramatic myelination defects in the CNS. Although proliferation is not altered in Seh1-deficient oligodendrocyte progenitor cells (OPCs), they fail to differentiate into mature oligodendrocytes, which impairs myelin production and remyelination after demyelinating injury...
March 2, 2019: Neuron
Martin Diebold, Tobias Derfuss
Background: Multiple sclerosis (MS) is an autoimmune demyelinating disorder of the central nervous system (CNS). Despite improvements of immunomodulatory therapies in relapsing-remitting MS, the pathomechanisms of progressive disease are poorly understood and therapeutically addressed to date. A pathophysiological role for proteins encoded by human endogenous retroviruses (HERVs) has been proposed. GNbAC1 is a monoclonal antibody directed against the envelope protein of a HERV with postulated involvement in MS...
2019: Therapeutic Advances in Neurological Disorders
Majid Ghareghani, Linda Scavo, Yahya Jand, Naser Farhadi, Hossein Sadeghi, Amir Ghanbari, Stefania Mondello, Damien Arnoult, Sajjad Gharaghani, Kazem Zibara
Metabolic disturbances have been implicated in demyelinating diseases including multiple sclerosis (MS). Melatonin, a naturally occurring hormone, has emerged as a potent neuroprotective candidate to reduce myelin loss and improve MS outcomes. In this study, we evaluated the effect of melatonin, at both physiological and pharmacological doses, on oligodendrocytes metabolism in an experimental autoimmune encephalomyelitis (EAE) mouse model of MS. Results showed that melatonin decreased neurological disability scores and enhanced remyelination, significantly increasing myelin protein levels including MBP, MOG, and MOBP...
2019: Frontiers in Pharmacology
Zhiqin Wang, Jianfeng Wang, Fang Yi, Lin Zhou, Yafang Zhou
Marchiafava-Bignami disease (MBD) is a rare condition characterized by demyelination, necrosis and atrophy of the corpus callosum (CC), and mainly associated with alcoholism. MBD may present with various clinical manifestations. Brain magnetic resonance imaging (MRI) scan is important in prompt diagnosis and treatment of MBD. Here we reported a case of MBD and reviewed literature about the usage of gadolinium-enhanced MRI in MBD. Gadolinium enhancement may indicate a condition at risk of developing necrosis...
2019: Frontiers in Human Neuroscience
Eva Leitzen, Barbara B Raddatz, Wen Jin, Sandra Goebbels, Klaus-Armin Nave, Wolfgang Baumgärtner, Florian Hansmann
Theiler's murine encephalomyelitis virus (TMEV)-induces a demyelinating disease in the spinal cord (SC) of susceptible but not in resistant (B6) mouse strains. The aim of the present study was to induce SC demyelination and a peripheral neuropathy in resistant mice by switching the infection site from cerebrum to SC. B6 mice were intraspinally inoculated with TMEV. Infected mice showed clinical signs starting at 7 days post infection (dpi). Histopathology revealed a mononuclear myelitis, centred on the injection site at 3 dpi with subsequent antero- and retrograde spread, accompanied by demyelination and axonal damage within the SC...
March 14, 2019: Scientific Reports
Jing Zhou, Yong-Chao Wu, Bao-Jun Xiao, Xiao-Dong Guo, Qi-Xin Zheng, Bin Wu
Demyelination of axons plays an important role in the pathology of many spinal cord diseases and injuries. Remyelination in demyelinated lesions is primarily performed by oligodendrocyte progenitor cells (OPCs), which generate oligodendrocytes in the developing and mature central nervous system. The efficiency of remyelination decreases with age. Many reports suggest that this decline in remyelination results from impaired OPC recruitment and differentiation during aging. Of the various molecular mechanisms involved in aging, changes in epigenetic modifications have received particular attention...
February 2019: Current Medical Science
Magnus Spangsberg Boesen, Maria J Miranda
Acquired demyelinating syndromes are inflammatory demyelinating CNS diseases. They can be either monophasic, such as acute disseminated encephalomyelitis (ADEM), or relapsing, such as multiple sclerosis (MS). In children, ADEM is more common before puberty, whereas MS becomes increasingly more frequent during puberty. This is a case report of a 13-year-old boy with acute onset of a spinal cord syndrome and possible encephalopathy. We discuss relevant diagnostic workup including testing for antibodies against myelin oligodendrocyte globulin and aquaporin-4, treatment, and risk of MS...
March 11, 2019: Ugeskrift for Laeger
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