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Spinal cord demyelination, cervical cord demyelination

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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
#1
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...
January 1, 2017: Neurorehabilitation and Neural Repair
https://www.readbyqxmd.com/read/28059798/sporadic-cases-with-novel-mutations-and-pedigree-in-hereditary-leukoencephalopathy-with-axonal-spheroids
#2
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...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28017200/the-characteristics-of-spinal-imaging-in-different-types-of-demyelinating-diseases
#3
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
#4
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 Sensory Neuropathy Lom Type (HMSNL), is an autosomal recessive, early-onset, severe demyelinating neuropathy with hearing loss, caused by 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. MRI showed slight atrophy of cerebellum, medulla oblongata and upper cervical spinal cord...
December 16, 2016: 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
#5
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...
December 8, 2016: Neurochemical Research
https://www.readbyqxmd.com/read/27872169/myelopathy-chameleons-and-mimics
#6
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
#7
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...
November 21, 2016: 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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27491693/a-compararison-of-magnetization-transfer-methods-to-assess-brain-and-cervical-cord-microstructure-in-multiple-sclerosis
#12
Alfonso Lema, Courtney Bishop, Omar Malik, Miriam Mattoscio, Rehiana Ali, Richard Nicholas, Paolo A Muraro, Paul M Matthews, Adam D Waldman, Rexford D Newbould
BACKGROUND: Demyelination is a core pathological feature of multiple sclerosis (MS) and spontaneous remyelination appears to be an important mechanism for repair in the disease. Magnetization transfer ratio imaging (MTR) has been used extensively to evaluate demyelination, although limitations to its specificity are recognized. MT saturation imaging (MTsat) removes some of the T1 dependence of MTR. We have performed a comparative evaluation of MTR and MTsat imaging in a mixed group of subjects with active MS, to explore their relative sensitivity to pathology relevant to explaining clinical outcomes...
August 5, 2016: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/27293119/venous-hypertensive-myelopathy-associated-with-cervical-spondylosis
#13
Seiji Okada, Charles Chang, Geraldine Chang, James J Yue
BACKGROUND CONTEXT: Venous hypertensive myelopathy (VHM) results from spinal vascular malformations of arteriovenous shunting that increases spinal venous pressure, leading to congestive edema and neurologic dysfunction. There has been no report of VHM associated with cervical spondylotic myelopathy (CSM). PURPOSE: The aim of this study was to report an extremely rare case of VHM likely due to CSM. STUDY DESIGN: This study is a case report and review of the literature...
June 9, 2016: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/27100385/tract-specific-and-age-related-variations-of-the-spinal-cord-microstructure-a-multi-parametric-mri-study-using-diffusion-tensor-imaging-dti-and-inhomogeneous-magnetization-transfer-ihmt
#14
Manuel Taso, Olivier M Girard, Guillaume Duhamel, Arnaud Le Troter, Thorsten Feiweier, Maxime Guye, Jean-Philippe Ranjeva, Virginie Callot
Being able to finely characterize the spinal cord (SC) microstructure and its alterations is a key point when investigating neural damage mechanisms encountered in different central nervous system (CNS) pathologies, such as multiple sclerosis, amyotrophic lateral sclerosis or myelopathy. Based on novel methods, including inhomogeneous magnetization transfer (ihMT) and dedicated SC probabilistic atlas post-processing, the present study focuses on the in vivo characterization of the healthy SC tissue in terms of regional microstructure differences between (i) upper and lower cervical vertebral levels and (ii) sensory and motor tracts, as well as differences attributed to normal aging...
June 2016: NMR in Biomedicine
https://www.readbyqxmd.com/read/26989546/acute-respiratory-failure-due-to-neuromyelitis-optica-treated-successfully-with-plasmapheresis
#15
Massa Zantah, Timothy B Coyle, Debapriya Datta
Neuromyelitis Optica (NMO) is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.
2016: Case Reports in Pulmonology
https://www.readbyqxmd.com/read/26410363/the-radiologically-isolated-syndrome
#16
REVIEW
C Lebrun
Even prior to the introduction of criteria defining the radiologically isolated syndrome (RIS), longitudinal clinical data from individuals with incidentally identified T2 lesions suggestive of multiple sclerosis (MS) were described. Healthy individuals who do not exhibit signs of neurological dysfunction may have a brain MRI performed for a reason other than suspicion of MS that reveals unexpected anomalies highly suggestive of demyelinating plaques given their size, location, and morphology. These healthy subjects lack a history or symptomatology suggestive of MS and fulfill formal criteria for RIS, a recently described MS subtype that shares the phenotype of at-risk individuals for future demyelinating events...
October 2015: Revue Neurologique
https://www.readbyqxmd.com/read/26383061/myelitis-differences-between-multiple-sclerosis-and-other-aetiologies
#17
S Presas-Rodríguez, L Grau-López, J V Hervás-García, A Massuet-Vilamajó, C Ramo-Tello
BACKGROUND: Myelitis can appear as an initial symptom in the context of demyelinating diseases, systemic inflammatory diseases, and infectious diseases. We aim to analyse the differences between myelitis associated with multiple sclerosis (MS) and myelitis resulting from other aetiologies. METHODS: Single-centre, retrospective analysis of patients with initial myelitis (2000-2013). Demographic, aetiological, clinical, radiological and prognostic variables were analysed and compared between patients with myelitis from MS and those with myelitis due to other aetiologies...
March 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/26381510/imaging-surrogates-of-disease-activity-in-neuromyelitis-optica-allow-distinction-from-multiple-sclerosis
#18
Lucy Matthews, Shannon Kolind, Alix Brazier, Maria Isabel Leite, Jonathan Brooks, Anthony Traboulsee, Mark Jenkinson, Heidi Johansen-Berg, Jacqueline Palace
Inflammatory demyelinating lesions of the central nervous system are a common feature of both neuromyelitis optica and multiple sclerosis. Despite this similarity, it is evident clinically that the accumulation of disability in patients with neuromyelitis optica is relapse related and that a progressive phase is very uncommon. This poses the question whether there is any pathological evidence of disease activity or neurodegeneration in neuromyelitis optica between relapses. To investigate this we conducted a longitudinal advanced MRI study of the brain and spinal cord in neuromyelitis optica patients, comparing to patients with multiple sclerosis and controls...
2015: PloS One
https://www.readbyqxmd.com/read/26378358/degenerative-cervical-myelopathy-a-spectrum-of-related-disorders-affecting-the-aging-spine
#19
REVIEW
Lindsay Tetreault, Christina L Goldstein, Paul Arnold, James Harrop, Alan Hilibrand, Aria Nouri, Michael G Fehlings
Cervical spinal cord dysfunction can result from either traumatic or nontraumatic causes, including tumors, infections, and degenerative changes. In this article, we review the range of degenerative spinal disorders resulting in progressive cervical spinal cord compression and propose the adoption of a new term, degenerative cervical myelopathy (DCM). DCM comprises both osteoarthritic changes to the spine, including spondylosis, disk herniation, and facet arthropathy (collectively referred to as cervical spondylotic myelopathy), and ligamentous aberrations such as ossification of the posterior longitudinal ligament and hypertrophy of the ligamentum flavum...
October 2015: Neurosurgery
https://www.readbyqxmd.com/read/26213113/clinical-manifestations-of-neuromyelitis-optica-in-male-and-female-patients
#20
Jianrui Yin, Youming Long, Fulan Shan, Yongxiang Fan, Linzhan Wu, Rong Zhong, Cong Gao, Xiaohui Chen, Qingchun Gao, Ning Yang
Neuromyelitis optica (NMO) is a severe, idiopathic, immuno-mediated, inflammatory demyelinating disease of the central nervous system. We examined the clinical features between male and female NMO patients, for which not much data exist. One hundred and eight Chinese Han patients with NMO were analysed retrospectively, all had been detected for the aquaporin-4 (AQP4) antibody using a cell-based assay. Of 108 NMO patients, 92 were female and 16 were male (female/male = 5.75). Ninety-four (87%) were positive for the AQP4 antibody in their serum and/or cerebral spinal fluid...
November 2015: Neurological Research
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