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demyelinating disorder

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https://www.readbyqxmd.com/read/29468668/myelin-oligodendrocyte-glycoprotein-and-aquaporin-4-antibodies-are-highly-specific-in-children-with-acquired-demyelinating-syndromes
#1
Sophie Duignan, Sukhvir Wright, Tom Rossor, John Cazabon, Kimberly Gilmour, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Cheryl Hemingway, Yael Hacohen
AIM: Our objectives were to evaluate the utility of measuring myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies (Ab) in clinical practice and describe their associated neurological phenotypes in children. METHOD: Between 2012 and 2017, 371 children with suspected acquired demyelinating syndromes (ADS) seen in three tertiary centres were tested for MOG-Ab and AQP4-Ab. Medical notes were retrospectively reviewed, and clinical and demographic data compiled...
February 22, 2018: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/29468179/cell-transplantation-strategies-for-acquired-and-inherited-disorders-of-peripheral-myelin
#2
A K M G Muhammad, Kevin Kim, Irina Epifantseva, Arwin Aghamaleky-Sarvestany, Megan E Simpkinson, Sharon Carmona, Jesse Landeros, Shaughn Bell, John Svaren, Robert H Baloh
Objective: To investigate transplantation of rat Schwann cells or human iPSC-derived neural crest cells and derivatives into models of acquired and inherited peripheral myelin damage. Methods: Primary cultured rat Schwann cells labeled with a fluorescent protein for monitoring at various times after transplantation. Human-induced pluripotent stem cells (iPSCs) were differentiated into neural crest stem cells, and subsequently toward a Schwann cell lineage via two different protocols...
February 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29467711/eye-movement-abnormalities-in-multiple-sclerosis-pathogenesis-modeling-and-treatment
#3
REVIEW
Alessandro Serra, Clara G Chisari, Manuela Matta
Multiple sclerosis (MS) commonly causes eye movement abnormalities that may have a significant impact on patients' disability. Inflammatory demyelinating lesions, especially occurring in the posterior fossa, result in a wide range of disorders, spanning from acquired pendular nystagmus (APN) to internuclear ophthalmoplegia (INO), among the most common. As the control of eye movements is well understood in terms of anatomical substrate and underlying physiological network, studying ocular motor abnormalities in MS provides a unique opportunity to gain insights into mechanisms of disease...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29459060/bickerstaff-s-brainstem-encephalitis-with-overlapping-guillain-barre-syndrome-usefulness-of-sequential-nerve-conduction-studies
#4
Choong Yi Fong, Hnin Wint Wint Aung, Arie Khairani, Chin Seng Gan, Nortina Shahrizaila, Khean Jin Goh
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP)...
February 16, 2018: Brain & Development
https://www.readbyqxmd.com/read/29455827/paraneoplastic-neuromyelitis-optica-spectrum-disorder-as-presentation-of-esophageal-adenocarcinoma
#5
Daniel C Wiener, Tamara B Kaplan, Carlos E Bravo-Iñiguez, Jordan Miller, Aaron L Berkowitz, Michael T Jaklitsch
Neuromyelitis optica spectrum disorders are a group of relapsing, inflammatory, demyelinating neurologic syndromes involving the central nervous system associated with antibodies against aquaporin-4. Although most commonly an idiopathic autoimmune condition, neuromyelitis optica may occur as a paraneoplastic syndrome in rare instances. We report a case of transverse myelitis caused by paraneoplastic neuromyelitis optica as the presenting clinical syndrome in a patient with esophageal adenocarcinoma.
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29455190/a-novel-paraneoplastic-syndrome-with-acquired-lipodystrophy-and-chronic-inflammatory-demyelinating-polyneuropathy-in-an-adolescent-male-with-craniopharyngioma
#6
Hillary Elizabeth Lockemer, Kathryn Maria Sumpter, Sandy Cope-Yokoyama, Abhimanyu Garg
BACKGROUND: Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. CASE PRESENTATION: A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP...
February 19, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29446144/animal-models-of-multiple-sclerosis-focus-on-experimental-autoimmune-encephalomyelitis
#7
REVIEW
Ivana Bjelobaba, Vesna Begovic-Kupresanin, Sanja Pekovic, Irena Lavrnja
Multiple sclerosis (MS) is a chronic, progressive disorder of the central nervous system (CNS) that affects more than two million people worldwide. Several animal models resemble MS pathology; the most employed are experimental autoimmune encephalomyelitis (EAE) and toxin- and/or virus-induced demyelination. In this review we will summarize our knowledge on the utility of different animal models in MS research. Although animal models cannot replicate the complexity and heterogeneity of the MS pathology, they have proved to be useful for the development of several drugs approved for treatment of MS patients...
February 15, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29445932/fibrodysplasia-ossificans-progressiva-a-current-review-of-imaging-findings
#8
REVIEW
Adam H Bauer, Jeff Bonham, Luis Gutierrez, Edward C Hsiao, Daria Motamedi
OBJECTIVE: Fibrodysplasia ossificans progressiva is a rare genetic disorder characterized by congenital skeletal deformities and soft tissue masses that progress to heterotopic ossification. Deformities of the great toes are distinctive, and heterotopic ossification in the soft tissues follows an expected anatomic and temporal pattern. In addition to heterotopic ossification, osteochondromata, middle ear ossification, demyelination, lymphedema, and venous thrombosis are characteristic...
February 14, 2018: Skeletal Radiology
https://www.readbyqxmd.com/read/29445493/acute-disseminated-encephalomyelitis-following-influenza-a-pneumonia
#9
Babikir Kheiri, Emad Abu Sitta, Anas Salih, Mohammed Al Qasmi, Ghassan Bachuwa
Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system and can present following influenza A infections as multifocal neurological deficits. ADEM remains a challenging diagnosis, and high clinical suspicious coupled with laboratory investigations and neuroimaging is required to exclude other primary and secondary demyelinating disorders.
February 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29445325/biomarkers-of-amyotrophic-lateral-sclerosis-current-status-and-interest-of-oxysterols-and-phytosterols
#10
REVIEW
Anne Vejux, Amira Namsi, Thomas Nury, Thibault Moreau, Gérard Lizard
Amyotrophic lateral sclerosis (ALS) is a non-demyelinating neurodegenerative disease in adults with motor disorders. Two forms exist: a sporadic form (90% of cases) and a family form due to mutations in more than 20 genes including the Superoxide dismutase 1, TAR DNA Binding Protein, Fused in Sarcoma, chromosome 9 open reading frame 72 and VAPB genes. The mechanisms associated with this pathology are beginning to be known: oxidative stress, glutamate excitotoxicity, protein aggregation, reticulum endoplasmic stress, neuroinflammation, alteration of RNA metabolism...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29445144/lenalidomide-regulates-cns-autoimmunity-by-promoting-m2-macrophages-polarization
#11
Qinjie Weng, Jiaying Wang, Jiajia Wang, Jing Wang, Fahmida Sattar, Zhikang Zhang, Jiahuan Zheng, Zijie Xu, Mengting Zhao, Xuan Liu, Lijun Yang, Guifeng Hao, Liang Fang, Q Richard Lu, Bo Yang, Qiaojun He
Multiple sclerosis (MS) is a chronic and debilitating neurological disorder of the central nervous system (CNS), characterized by infiltration of leukocytes into CNS and subsequent demyelination. Emerging evidences have revealed the beneficial roles of M2 macrophages in ameliorating experimental autoimmune encephalomyelitis (EAE), a model for MS. Here, we identify that lenalidomide alone could promote macrophages M2 polarization to prevent the progression of EAE, which is associated with subsequent inhibition of proinflammatory Th1 and Th17 cells both in peripheral lymph system and CNS...
February 14, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29444299/early-nodal-and-paranodal-disruption-in-autoimmune-optic-neuritis
#12
Aleksandar Stojic, Jovana Bojcevski, Sarah K Williams, Ricarda Diem, Richard Fairless
Disturbances in the nodes of Ranvier are an early phenomenon in many CNS disorders, including the autoimmune demyelinating disease multiple sclerosis (MS). Using an animal model of optic neuritis, a common early symptom of MS, we have investigated nodal and paranodal compartments in the optic nerve during disease progression. Both nodes and paranodes, as identified by immunohistochemistry against sodium channels (Nav) and Caspr, respectively, were observed to increase in length during the late induction phase of the disease, prior to onset of the demyelination and immune cell infiltration characteristic of optic neuritis...
February 10, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29443442/paediatric-adem-followed-by-optic-neuritis-disease-course-treatment-response-and-outcome
#13
Y Y M Wong, Y Hacohen, T Armangue, E Wassmer, H Verhelst, C Hemingway, E D van Pelt, C E Catsman-Berrevoets, R Q Hintzen, K Deiva, M J Lim, K Rostásy, R F Neuteboom
BACKGROUND: Acute disseminated encephalomyelitis, followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome different than MS and neuromyelitis optica spectrum disorder. We aim to describe the disease course, treatment response and outcome of these children. METHODS: children <18 years were identified from 6 countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM, followed by at least one ON...
February 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29443059/in-vivo-electrophysiological-measurement-of-the-rat-ulnar-nerve-with-axonal-excitability-testing
#14
Brandon M Wild, Renée Morris, Mihai Moldovan, Christian Krarup, Arun V Krishnan, Ria Arnold
Electrophysiology enables the objective assessment of peripheral nerve function in vivo. Traditional nerve conduction measures such as amplitude and latency detect chronic axon loss and demyelination, respectively. Axonal excitability techniques "by threshold tracking" expand upon these measures by providing information regarding the activity of ion channels, pumps and exchangers that relate to acute function and may precede degenerative events. As such, the use of axonal excitability in animal models of neurological disorders may provide a useful in vivo measure to assess novel therapeutic interventions...
February 6, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29438836/mog-antibody-demyelinating-diseases-a-case-of-post-partum-severe-rhombencephalitis-and-transverse-myelitis
#15
D Vecchio, E Virgilio, P Naldi, C Comi, R Cantello
INTRODUCTION: Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) associated disorders present with a spectrum of clinical pictures including brainstem involvement. CASE REPORT: A patient with the sudden onset of a post-partum severe rhombencephalitis causing respiratory failure (12 years after a mild transverse myelitis). Despite the aggressive clinical course, she had an impressive recovery after plasmapheresis, and no further relapses on immunosuppression...
February 8, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29438266/new-ways-of-seeing-the-mechanistic-heterogeneity-of-multiple-sclerosis-plaque-pathogenesis
#16
Ethan I Meltzer, Fiona E Costello, Elliot M Frohman, Teresa C Frohman
BACKGROUND: Over the past few decades, we have witnessed a transformation with respect to the principles and pathobiological underpinnings of multiple sclerosis (MS). From the traditional rubric of MS as an inflammatory and demyelinating disorder restricted to central nervous system (CNS) white matter, our contemporary view has evolved to encompass a broader understanding of the variable mechanisms that contribute to tissue injury, in a disorder now recognized to affect white and grey matter compartments...
March 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29434480/a-rare-presentation-of-neuromyelitis-optica-spectrum-disorders
#17
Navneet K Singh, Alexander J Sweidan, Sarah Strube, Ignacio Carrillo-Nunez
Neuromyelitis optica spectrum disorders (NMOSDs) are a set of demyelinating disorders that primarily target the optic nerves and the spinal cord. Previously thought to be a subset of multiple sclerosis (MS), now is recognized as a distinct entity. We present a 59-year-old female patient who was admitted for acute upper and lower extremity weakness. The patient had woken up from sleep with sudden onset of weakness. Patient was initially diagnosed with a right hemispheric stroke; however, magnetic resonance imaging of the cervical spine later performed showed abnormal enhancement from C2-C4, representing transverse myelitis...
2018: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29433112/-zika-virus-and-guillain-barr%C3%A3-syndrome
#18
Haruki Koike
Recent studies revealed an association between Zika virus infection and a variety of neurological disorders, including microcephaly, meningoencephalitis, myelitis, and Guillain-Barré syndrome (GBS). Following the first report of a patient diagnosed with GBS after Zika virus infection in December 2013, the number of GBS patients significantly increased in endemic countries, such as French-Polynesia and Latin American countries. Electrophysiological studies suggested that patients suffering from GBS associated with Zika virus infection manifest acute inflammatory demyelinating polyneuropathy (AIDP), rather than acute motor axonal neuropathy (AMAN)...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29432441/fas-promoter-polymorphisms-and-serum-sfas-level-are-associated-with-increased-risk-of-nerve-damage-in-bangladeshi-patients-with-guillain-barr%C3%A3-syndrome
#19
Zhahirul Islam, Israt Jahan, Rijwan U Ahammad, Mohammad Shahnaij, Shamsun Nahar, Quazi D Mohammad
Guillain-Barré syndrome (GBS) is an autoimmune disorder of the peripheral nervous system triggered by molecular mimicry between pathogen lipopolysaccharides and host nerve gangliosides. Polymorphisms in the Fas receptor (FAS) and Fas ligand (FASL) genes may potentially alter the elimination of autoreactive immune cells and affect disease susceptibility or disease severity in GBS. We detected single nucleotide polymorphisms (SNPs) in FAS (-1377G/A and -670A/G) and FASL (-843C/T) in a prospective cohort of 300 patients with GBS and 300 healthy controls from the Bangladeshi population...
2018: PloS One
https://www.readbyqxmd.com/read/29428821/recombinant-igg1-fc-hexamers-block-cytotoxicity-and-pathological-changes-in-experimental-in-vitro-and-rat-models-of-neuromyelitis-optica
#20
Lukmanee Tradtrantip, Christian M Felix, Rolf Spirig, Adriana Baz Morelli, A S Verkman
Intravenous human immunoglobulin G (IVIG) may have therapeutic benefit in neuromyelitis optica spectrum disorders (herein called NMO), in part because of the anti-inflammatory properties of the IgG Fc region. Here, we evaluated recombinant Fc hexamers consisting of the IgM μ-tailpiece fused with the Fc region of human IgG1. In vitro, the Fc hexamers prevented cytotoxicity in aquaporin-4 (AQP4) expressing cells and in rat spinal cord slice cultures exposed to NMO anti-AQP4 autoantibody (AQP4-IgG) and complement, with >500-fold greater potency than IVIG or monomeric Fc fragments...
February 8, 2018: Neuropharmacology
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