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Spinal cord demyelination, cervical cord demyelination

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https://www.readbyqxmd.com/read/28725551/cervical-cord-myelin-water-imaging-shows-degenerative-changes-over-one-year-in-multiple-sclerosis-but-not-neuromyelitis-optica-spectrum-disorder
#1
Anna J E Combes, Lucy Matthews, Jimmy S Lee, David K B Li, Robert Carruthers, Anthony L Traboulsee, Gareth J Barker, Jacqueline Palace, Shannon Kolind
Spinal cord pathology is a feature of both neuromyelitis optica spectrum disorder (NMOSD) and relapsing-remitting multiple sclerosis (MS). While subclinical disease activity has been described in MS using quantitative magnetic resonance imaging measures, current evidence suggests that neurodegeneration is absent between relapses in NMOSD, although most evidence comes from brain studies. We aimed to assess cross-sectional differences and longitudinal changes in myelin integrity in relapse-free MS and NMOSD subjects over one year...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28716281/-black-butterfly-sign-on-t2-weighted-and-susceptibility-weighted-imaging-a-novel-finding-of-chronic-venous-congestion-of-the-brain-stem-and-spinal-cord-associated-with-dural-arteriovenous-fistulas
#2
Mikako Enokizono, Noriko Sato, Minoru Morikawa, Yukio Kimura, Atsuhiko Sugiyama, Tomoko Maekawa, Daichi Sone, Daiki Takewaki, Tomoko Okamoto, Yuji Takahashi, Nobutaka Horie, Takayuki Matsuo
A dural arteriovenous fistula (DAVF) with spinal perimedullary venous drainage can cause progressive myelopathy, and it is sometimes incorrectly diagnosed as another spinal cord disease. Here we report the cases of three individuals with a DAVF (one craniocervical junction DAVF and two tentorial DAVFs) with progressive myelopathy showing unique magnetic resonance (MR) imaging findings. MR T2*WI or susceptibility-weighted imaging (SWI) demonstrated symmetrical dark signal intensity lesions predominantly in the dorsal aspect of medulla and the central gray matter of cervical spinal cord that showed the "black butterfly" silhouette...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28701500/assessing-structure-and-function-of-myelin-in-cervical-spondylotic-myelopathy-evidence-of-demyelination
#3
Hanwen Liu, Erin L MacMillian, Catherine R Jutzeler, Emil Ljungberg, Alex L MacKay, Shannon H Kolind, Burkhard Mädler, David K B Li, Marcel F Dvorak, Armin Curt, Cornelia Laule, John L K Kramer
PURPOSE: To assess the extent of demyelination in cervical spondylotic myelopathy (CSM) using myelin water imaging (MWI) and electrophysiologic techniques. METHODS: Somatosensory evoked potentials (SSEPs) and MWI were acquired in 14 patients with CSM and 18 age-matched healthy controls. MWI was performed on a 3.0T whole body magnetic resonance scanner. Myelin water fraction (MWF) was extracted for the dorsal columns and whole cord. SSEPs and MWF were also compared with conventional MRI outcomes, including T2 signal intensity, compression ratio, maximum spinal cord compression (MSCC), and maximum canal compromise (MCC)...
August 8, 2017: Neurology
https://www.readbyqxmd.com/read/28646336/nogo-a-antibodies-enhance-axonal-repair-and-remyelination-in-neuro-inflammatory-and-demyelinating-pathology
#4
Benjamin V Ineichen, Sandra Kapitza, Christiane Bleul, Nicolas Good, Patricia S Plattner, Maryam S Seyedsadr, Julia Kaiser, Marc P Schneider, Björn Zörner, Roland Martin, Michael Linnebank, Martin E Schwab
Two hallmarks of chronic multiple sclerosis lesions are the absence of significant spontaneous remyelination and primary as well as secondary neurodegeneration. Both characteristics may be influenced by the presence of inhibitory factors preventing myelin and neuronal repair. We investigated the potential of antibodies against Nogo-A, a well-known inhibitory protein for neuronal growth and plasticity, to enhance neuronal regeneration and remyelination in two animal models of multiple sclerosis. We induced a targeted experimental autoimmune encephalomyelitis (EAE) lesion in the dorsal funiculus of the cervical spinal cord of adult rats resulting in a large drop of skilled forelimb motor functions...
June 23, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28619424/cerebrospinal-fluid-and-serum-levels-of-interleukin-8-in-patients-with-multiple-sclerosis-and-its-correlation-with-q-albumin
#5
Z Matejčíková, J Mareš, V Sládková, T Svrčinová, J Vysloužilová, J Zapletalová, P Kaňovský
INTRODUCTION: Multiple sclerosis (MS) is an inflammatory-demyelinating disease of the central nervous system (CNS). Autoimmune inflammation is common in the early stages of MS and is followed by neurodegenerative processes. The result of these changes is axon and myelin breakdown. The paraclinical examination methods are an important part of the diagnostic process. Magnetic resonance imaging of the brain and the cervical spinal cord and an examination of cerebrospinal fluid (CSF) are common paraclinical examinations...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28420330/anti-mog-antibody-positive-adem-following-infectious-mononucleosis-due-to-a-primary-ebv-infection-a-case-report
#6
Yoshitsugu Nakamura, Hideto Nakajima, Hiroki Tani, Takafumi Hosokawa, Shimon Ishida, Fumiharu Kimura, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima
BACKGROUND: Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein-Barr virus (EBV) infection. CASE PRESENTATION: A 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#7
Natalia Petrova, Daniele Carassiti, Daniel R Altmann, David Baker, Klaus Schmierer
Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area (CSA) assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between CSA and axonal loss, and their correlation with demyelination, have never been systematically investigated using human post mortem tissue...
April 12, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28358726/local-delivery-of-thyroid-hormone-enhances-oligodendrogenesis-and-myelination-after-spinal-cord-injury
#8
Robert B Shultz, Zhicheng Wang, Jia Nong, Zhiling Zhang, Yinghui Zhong
OBJECTIVE: Traumatic spinal cord injury (SCI) causes apoptosis of myelin-forming oligodendrocytes (OLs) and demyelination of surviving axons, resulting in conduction failure. Remyelination of surviving denuded axons provides a promising therapeutic target for spinal cord repair. While cell transplantation has demonstrated efficacy in promoting remyelination and functional recovery, the lack of ideal cell sources presents a major obstacle to clinical application. The adult spinal cord contains oligodendrocyte precursor cells and multipotent neural stem/progenitor cells that have the capacity to differentiate into mature, myelinating OLs...
June 2017: Journal of Neural Engineering
https://www.readbyqxmd.com/read/28212662/marked-central-nervous-system-pathology-in-cd59-knockout-rats-following-passive-transfer-of-neuromyelitis-optica-immunoglobulin-g
#9
Xiaoming Yao, Alan S Verkman
Neuromyelitis optica spectrum disorders (herein called NMO) is an inflammatory demyelinating disease of the central nervous system in which pathogenesis involves complement-dependent cytotoxicity (CDC) produced by immunoglobulin G autoantibodies targeting aquaporin-4 (AQP4-IgG) on astrocytes. We reported evidence previously, using CD59(-/-) mice, that the membrane-associated complement inhibitor CD59 modulates CDC in NMO (Zhang and Verkman, J. Autoimmun. 53:67-77, 2014). Motivated by the observation that rats, unlike mice, have human-like complement activity, here we generated CD59(-/-) rats to investigate the role of CD59 in NMO and to create NMO pathology by passive transfer of AQP4-IgG under conditions in which minimal pathology is produced in normal rats...
February 17, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28107804/clinically-relevant-levels-of-4-aminopyridine-strengthen-physiological-responses-in-intact-motor-circuits-in-rats-especially-after-pyramidal-tract-injury
#10
Anil Sindhurakar, Asht M Mishra, Disha Gupta, Jennifer F Iaci, Tom J Parry, Jason B Carmel
BACKGROUND: 4-Aminopyridine (4-AP) is a Food and Drug Administration-approved drug to improve motor function in people with multiple sclerosis. Preliminary results suggest the drug may act on intact neural circuits and not just on demyelinated ones. OBJECTIVE: To determine if 4-AP at clinically relevant levels alters the excitability of intact motor circuits. METHODS: In anesthetized rats, electrodes were placed over motor cortex and the dorsal cervical spinal cord for electrical stimulation, and electromyogram electrodes were inserted into biceps muscle to measure responses...
April 2017: Neurorehabilitation and Neural Repair
https://www.readbyqxmd.com/read/28059798/sporadic-cases-with-novel-mutations-and-pedigree-in-hereditary-leukoencephalopathy-with-axonal-spheroids
#11
Liyong Wu, Jia Liu, Longze Sha, Xianling Wang, Jieying Li, Jing Dong, Jianping Jia
Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal dominant hereditary disease, featured by cerebral white matter degeneration with demyelination and axonal spheroids. We collected three gene-confirmed HDLS cases in our neurodegenerative clinic. Two HDLS cases were sporadic with novel mutations, while another case had a family history with previously described mutations. All three cases suffered memory problems with white matter lesions and pyramid signs. No obvious clinical differences were observed between sporadic and familial HDLS cases...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28017200/the-characteristics-of-spinal-imaging-in-different-types-of-demyelinating-diseases
#12
COMPARATIVE STUDY
Chaisak Dumrikarnlert, Sasitorn Siritho, Pimwalai Chulapimphan, Chanon Ngamsombat, Chanjira Satukijchai, Naraporn Prayoonwiwat
BACKGROUND: Transverse myelitis is the common presentation in demyelinating conditions. OBJECTIVE: To determine the characteristics of spinal lesions among each type of demyelinating diseases. METHODS: Medical records and spinal imaging of patients who were [1] older than 18years, [2] had at least one attack of TM, [3] had available spinal MRI data and [4] were tested for aquaporin-4 antibody were included. RESULTS: One hundred and fifty-eight patients were eligible (27 clinically isolated syndrome [CIS], 38 MS, 55 seropositive neuromyelitis optica spectrum disorders [NMOSD], 9 seronegative NMOSD, and 29 idiopathic transverse myelitis [IDD-TM])...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27982524/a-novel-ndrg1-mutation-in-a-non-romani-patient-with-cmt4d-hmsn-lom
#13
Giuseppe Piscosquito, Stefania Magri, Paola Saveri, Micaela Milani, Claudia Ciano, Laura Farina, Franco Taroni, Davide Pareyson
Charcot-Marie-Tooth disease type 4D (CMT4D), also known as hereditary motor and sensory neuropathy Lom type (HMSNL), is an autosomal recessive, early onset, severe demyelinating neuropathy with hearing loss, caused by N-Myc downstream-regulated gene 1 (NDRG1) mutations. CMT4D is rare with only three known mutations, one of which (p.Arg148Ter) is found in patients of Romani ancestry and accounts for the vast majority of cases. We report a 38-year-old Italian female with motor development delay, progressive neuropathy, and sensorineural deafness...
March 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/27933549/demyelination-occurred-as-the-secondary-damage-following-diffuse-axonal-loss-in-a-rat-model-of-radiation-myelopathy
#14
Li Wei, Yong Zhou, Chang-Jiang Liu, Ke Zheng, Hua You
The main purpose of the present study was to examine the time and dose-dependent course of demyelination in the rat radiation myelopathy model in the first 180 days after irradiation of the spinal cord. An irradiated cervical spinal cord rat model (C2-T2 segment) was generated using a (60)Co irradiator to deliver 50 Gy and 100 Gy, respectively. The behavioral dysfunction was observed by the forelimb paralysis scoring system. The histological damage in the irradiated spinal cord was examined by hematoxylin/eosin staining, luxol fast blue staining, immunohistochemical analysis, methylene blue/Azure II staining, and uranyl/lead salts staining...
April 2017: Neurochemical Research
https://www.readbyqxmd.com/read/27872169/myelopathy-chameleons-and-mimics
#15
Lionel Ginsberg
The diagnosis of spinal cord disease may be delayed or missed if the presentation does not conform to the expected pattern of a symmetrical spastic paraparesis with sphincter dysfunction and a sensory level. This may occur when a myelopathy has yet to evolve fully, or is highly asymmetrical, as in Brown-Séquard syndrome. Other potential distractions include fluctuating symptoms, as may accompany spinal cord demyelination, and pseudoneuropathic features, as seen acutely in spinal shock and in the chronic setting with some high cervical cord lesions...
January 2017: Practical Neurology
https://www.readbyqxmd.com/read/27830953/radiologically-isolated-syndrome-watchful-waiting-vs-active-treatment
#16
REVIEW
Foziah Alshamrani, Hind Alnajashi, M Freedman
Neurologists are frequently consulted for patients who have white matter lesions discovered incidentally on their brain MRI, performed for reasons other than suspecting a demyelinating disease. The referring physician will question if the person has multiple sclerosis (MS). If the MRI is typical for MS but there are no clinical symptoms or signs suggestive of a demyelinating disorder, patients are diagnosed as having a Radiologically Isolated Syndrome (RIS). Areas covered: This is a timely review on RIS with a focus on treatment considerations...
May 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/27690115/morphology-of-donor-and-recipient-nerves-utilised-in-nerve-transfers-to-restore-upper-limb-function-in-cervical-spinal-cord-injury
#17
Aurora Messina, Natasha Van Zyl, Michael Weymouth, Stephen Flood, Andrew Nunn, Catherine Cooper, Jodie Hahn, Mary P Galea
Loss of hand function after cervical spinal cord injury (SCI) impacts heavily on independence. Multiple nerve transfer surgery has been applied successfully after cervical SCI to restore critical arm and hand functions, and the outcome depends on nerve integrity. Nerve integrity is assessed indirectly using muscle strength testing and intramuscular electromyography, but these measures cannot show the manifestation that SCI has on the peripheral nerves. We directly assessed the morphology of nerves biopsied at the time of surgery, from three patients within 18 months post injury...
September 27, 2016: Brain Sciences
https://www.readbyqxmd.com/read/27664830/g-ratio-weighted-imaging-of-the-human-spinal-cord-in-vivo
#18
T Duval, S Le Vy, N Stikov, J Campbell, A Mezer, T Witzel, B Keil, V Smith, L L Wald, E Klawiter, J Cohen-Adad
The fiber g-ratio is defined as the ratio of the inner to the outer diameter of the myelin sheath. This ratio provides a measure of the myelin thickness that complements axon morphology (diameter and density) for assessment of demyelination in diseases such as multiple sclerosis. Previous work has shown that an aggregate g-ratio map can be computed using a formula that combines axon and myelin density measured with quantitative MRI. In this work, we computed g-ratio weighted maps in the cervical spinal cord of nine healthy subjects...
January 15, 2017: NeuroImage
https://www.readbyqxmd.com/read/27638926/progressive-solitary-sclerosis-gradual-motor-impairment-from-a-single-cns-demyelinating-lesion
#19
B Mark Keegan, Timothy J Kaufmann, Brian G Weinshenker, Orhun H Kantarci, William F Schmalstieg, M Mateo Paz Soldan, Eoin P Flanagan
OBJECTIVE: To report patients with progressive motor impairment resulting from an isolated CNS demyelinating lesion in cerebral, brainstem, or spinal cord white matter that we call progressive solitary sclerosis. METHODS: Thirty patients were identified with (1) progressive motor impairment for over 1 year with a single radiologically identified CNS demyelinating lesion along corticospinal tracts, (2) absence of other demyelinating CNS lesions, and (3) no history of relapses affecting other CNS pathways...
October 18, 2016: Neurology
https://www.readbyqxmd.com/read/27538633/cervical-demyelinating-lesion-presenting-with-choreoathetoid-movements-and-dystonia
#20
Silvia de Pasqua, Sabina Cevoli, Fabio Calbucci, Rocco Liguori
Pseudoathetosis and dystonia are rare manifestations of spinal cord disease that have been already reported in lesions involving the posterior columns at the cervical level. We report two patients with a cervical demyelinating lesion at C3-C4 level presenting with hand dystonia and pseudoathetoid movements. The movement disorder disappeared after steroid treatment. The cases we described highlight the importance of identifying secondary causes of movement disorders that can be reversible with appropriate therapy...
September 15, 2016: Journal of the Neurological Sciences
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