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https://www.readbyqxmd.com/read/29032462/association-of-tnfsf4-polymorphisms-with-neuromyelitis-optica-spectrum-disorders-in-a-chinese-population
#1
Zhiyun Lian, Ju Liu, Ziyan Shi, Hongxi Chen, Qin Zhang, Huiru Feng, Qin Du, Xiaohui Miao, Hongyu Zhou
The tumor necrosis factor ligand superfamily member 4 (TNFSF4) gene encodes a vital co-stimulatory molecule of the immune system and has been identified as a susceptibility locus for systemic lupus erythematosus, systemic sclerosis, and primary Sjögren's syndrome. However, the association of TNFSF4 polymorphisms with neuromyelitis optica spectrum disorders (NMOSD), an inflammatory, demyelinating autoimmune disease of the central nervous system, has not yet been investigated. To evaluate whether TNFSF4 polymorphisms contribute to risk of NMOSD, four single-nucleotide polymorphisms (SNPs) (rs1234315, rs2205960, rs704840, and rs844648) were selected and genotyped in a cohort of 312 patients with NMOSD and 487 healthy controls...
October 14, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/29027647/dasatinib-associated-reversible-demyelinating-peripheral-polyneuropathy-in-a-case-of-chronic-myeloid-leukemia
#2
Takashi Ishida, Naoyuki Akagawa, Tomomi Miyata, Naomi Tominaga, Takahiro Iizuka, Masaaki Higashihara, Takahiro Suzuki, Koji Miyazaki
Tyrosine kinase inhibitors (TKIs) are essential for the treatment of chronic myeloid leukemia (CML). Adverse effects of dasatinib have been reported; however, few reports have highlighted the association between dasatinib and demyelinating peripheral neuropathy (DPN). We report a patient with CML who developed acute onset of DPN associated with dasatinib therapy. A 46-year-old Japanese woman was treated with dasatinib for 7 months after the diagnosis of CML and she achieved a major molecular response (MMR)...
October 13, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/29026634/hiv-positive-patient-with-gbs-like-syndrome
#3
Samantha J Shepherd, Heather Black, Emma C Thomson, Rory N Gunson
Introduction. Guillain-Barré Syndrome (GBS) is an acute demyelinating polyneuropathy which can occur post-infection. Criteria of diagnosis of GBS include areflexia with progressive bilateral weakness in arms and legs. GBS can lead to severe respiratory and cardiac complications. The fatality rate can be up to 5 % in patients, depending on the severity of the symptoms. HIV can cause a range of neurological disorders including, on rare occasions, GBS. GBS can occur at any stage of HIV infection, highlighting the complexity of diagnosis of GBS within HIV patients...
August 2017: JMM Case Reports
https://www.readbyqxmd.com/read/29026001/understanding-a-role-for-hypoxia-in-lesion-formation-and-location-in-the-deep-and-periventricular-white-matter-in-small-vessel-disease-and-multiple-sclerosis
#4
REVIEW
Santiago Martinez Sosa, Kenneth J Smith
The deep and periventricular white matter is preferentially affected in several neurological disorders, including cerebral small vessel disease (SVD) and multiple sclerosis (MS), suggesting that common pathogenic mechanisms may be involved in this injury. Here we consider the potential pathogenic role of tissue hypoxia in lesion development, arising partly from the vascular anatomy of the affected white matter. Specifically, these regions are supplied by a sparse vasculature fed by long, narrow end arteries/arterioles that are vulnerable to oxygen desaturation if perfusion is reduced (as in SVD, MS and diabetes) or if the surrounding tissue is hypoxic (as in MS, at least)...
October 15, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/29025774/posterior-fossa-progressive-multifocal-leukoencephalopathy-first-presentation-of-an-unknown-autoimmune-disease
#5
Paulette Scholten, Peter Kralt, Bram Jacobs
We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous system, caused by an opportunistic infection with John Cunningham virus. PML has previously been considered a lethal condition, but because of careful monitoring of patients with HIV and of patients using immunosuppressive drugs it is discovered in earlier stages and prognosis can be improved...
October 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28990564/-mirnas-new-actors-in-the-physiopathology-of-multiple-sclerosis
#6
Ferdinand Jagot, Nathalie Davoust
Multiple sclerosis (MS) is an auto-immune demyelinating disorder characterized by a chronic neuro-inflammatory process associated with an infiltration of the central nervous system (CNS) by autoreactive lymphocytes. The etiology of the disease remains unclear but the recent discovery of a dysregulated miRNA network in both cells and extracellular fluids of MS patients has brought new insights on the pathophysiological mechanisms involved in this disorder. miRNAs can induce a T cell polarization towards a pathological Th17 or Th1 phenotype and a deleterious activation of microglia, the CNS-resident macrophages...
June 2017: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28990252/differential-brainstem-atrophy-patterns-in-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorders
#7
Chi-Yan Lee, Henry Ka-Fung Mak, Pui-Wai Chiu, Hing-Chiu Chang, Frederik Barkhof, Koon-Ho Chan
BACKGROUND: Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are central nervous system (CNS) inflammatory demyelinating disorders. It is clinically important to distinguish MS from NMOSD, as treatment and prognosis differ. Brainstem involvement is common in both disorders. PURPOSE: To investigate whether the patterns of brainstem atrophy on volumetric analysis in MS and NMOSD were different and correlated with clinical disability. STUDY TYPE: Case-control cross-sectional study...
October 9, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28988734/intractable-nausea-due-to-the-area-postrema-syndrome-of-neuromyelitis-optica-an-uncommon-cause-of-a-common-symptom
#8
Allison Snyder, Angela D Smedley, Stephen G Reich
BACKGROUND: Nausea and vomiting are common emergency department (ED) complaints. Neuromyelitis optica, a demyelinating disorder, has a predilection for the area postrema, the central nausea and vomiting center. Demyelinating lesions in this region cause intractable nausea and vomiting. CASE REPORT: We present a case of area postrema syndrome due to neuromyelitis optica in a 34-year-old woman who was seen in several EDs before the appropriate diagnosis was made. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Nausea and vomiting are complaints that commonly bring people to the ED, thus, emergency physicians are likely to be the first to encounter and diagnose the area postrema syndrome...
October 5, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28987189/diseases-of-the-peripheral-nerves
#9
Istvan Katona, Joachim Weis
This chapter reviews the diseases of the peripheral nerves from a neuropathologic point of view, with a special focus on specific morphologic changes, and includes a summary of the histopathologic methods available for their diagnosis. As the rate of obesity and the prevalence of type 2 diabetes increase, diabetic neuropathy is the most common cause of peripheral neuropathy. Many systemic disorders with metabolic origin, like amyloidosis, hepatic failure, vitamin deficiencies, uremia, lipid metabolism disorders, and others, can also cause axonal or myelin alterations in the peripheral nervous system...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28987175/inflammatory-demyelinating-diseases-of-the-central-nervous-system
#10
Romana Höftberger, Hans Lassmann
Inflammatory demyelinating diseases are a heterogeneous group of disorders, which occur against the background of an acute or chronic inflammatory process. The pathologic hallmark of multiple sclerosis (MS) is the presence of focal demyelinated lesions with partial axonal preservation and reactive astrogliosis. Demyelinated plaques are present in the white as well as gray matter, such as the cerebral or cerebellar cortex and brainstem nuclei. Activity of the disease process is reflected by the presence of lesions with ongoing myelin destruction...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28984582/multiple-system-atrophy-an%C3%A2-oligodendroglioneural-synucleinopathy
#11
Kurt A Jellinger
Multiple system atrophy (MSA) is an orphan, fatal, adult-onset neurodegenerative disorder of uncertain etiology that is clinically characterized by various combinations of parkinsonism, cerebellar, autonomic, and motor dysfunction. MSA is an α-synucleinopathy with specific glioneuronal degeneration involving striatonigral, olivopontocerebellar, and autonomic nervous systems but also other parts of the central and peripheral nervous systems. The major clinical variants correlate with the morphologic phenotypes of striatonigral degeneration (MSA-P) and olivopontocerebellar atrophy (MSA-C)...
September 26, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28976217/drugs-approved-for-the-treatment-of-multiple-sclerosis-review-of-their-safety-profile
#12
Fabiana Auricchio, Cristina Scavone, Daniela Cimmaruta, Gabriella di Mauro, Annalisa Capuano, Liberata Sportiello, Concetta Rafaniello
Multiple sclerosis (MS) is a chronic immune-mediated inflammatory disorder of the brain and spinal cord characterized by inflammation, demyelination, and axonal degeneration. Area covered: Even though the pharmacological armamentarium for MS treatment is considerably improved in the last 20 years, safety data especially for the second-line and innovative treatments are lacking. In order to analyze the safety profile of drugs used for the treatment of MS, a literature review of pre-marketing, post-marketing studies and case reports was performed...
October 4, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28968364/chronic-demyelinating-polyneuropathies
#13
Jeffrey A Allen
PURPOSE OF REVIEW: This article reviews the chronic demyelinating neuropathies, with a focus on the diagnosis and treatment of immune-mediated neuropathies and the features that can help differentiate immune-mediated neuropathies from other chronic demyelinating peripheral nerve conditions. RECENT FINDINGS: Advances in clinical phenotyping and outcomes assessment have enabled neurologists to improve disease recognition, treatment, and disease monitoring. Our understanding of the immunopathogenesis of demyelinating neuropathies is evolving...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28966634/lingo-1-and-amigo3-potential-therapeutic-targets-for-neurological-and-dysmyelinating-disorders
#14
REVIEW
Simon Foale, Martin Berry, Ann Logan, Daniel Fulton, Zubair Ahmed
Leucine rich repeat proteins have gained considerable interest as therapeutic targets due to their expression and biological activity within the central nervous system. LINGO-1 has received particular attention since it inhibits axonal regeneration after spinal cord injury in a RhoA dependent manner while inhibiting leucine rich repeat and immunoglobulin-like domain-containing protein 1 (LINGO-1) disinhibits neuron outgrowth. Furthermore, LINGO-1 suppresses oligodendrocyte precursor cell maturation and myelin production...
August 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28964929/towards-quantification-of-myelin-by-solid-state-mri-of-the-lipid-matrix-protons
#15
Alan C Seifert, Cheng Li, Michael J Wilhelm, Suzanne L Wehrli, Felix W Wehrli
PURPOSE: Direct assessment of myelin has the potential to reveal central nervous system abnormalities and serve as a means to follow patients with demyelinating disorders during treatment. Here, we investigated the feasibility of direct imaging and quantification of the myelin proton pool, without the many possible confounds inherent to indirect methods, via long-T2 suppressed 3D ultra-short echo-time (UTE) and zero echo-time (ZTE) MRI in ovine spinal cord. METHODS: ZTE and UTE experiments, with and without inversion-recovery (IR) preparation, were conducted in ovine spinal cords before and after D2O exchange of tissue water, on a 9...
September 28, 2017: NeuroImage
https://www.readbyqxmd.com/read/28961956/tremor-secondary-to-a-thalamic-glioma-a-case-report
#16
Tony R Wang, Camilo E Fadul, W Jeff Elias
BACKGROUND AND IMPORTANCE: Tremor is the most prevalent movement disorder. While the exact pathophysiology remains to be elucidated, the importance of the thalamus in tremor circuitry is well recognized. Thalamic lesions from demyelination, trauma, ischemia, or neoplasm rarely cause isolated tremor. We report the case of a patient presenting with a tremor secondary to a thalamic grade II astrocytoma that improved with treatment. CLINICAL PRESENTATION: A 50-yr-old male presented with a 1-yr history of right-hand tremor...
August 5, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/28954972/steroid-responsive-demyelinating-peripheral-neuropathy-associated-with-chronic-lymphoproliferative-disorders-of-natural-killer-cells
#17
Hironori Sano, Toshihiko Maeda, Masatoshi Omoto, Jun-Ichi Ogasawara, Michiaki Koga, Motoharu Kawai, Takashi Kanda
We herein report the findings of a 67-year-old woman with steroid-responsive multiple mononeuropathy associated with chronic natural killer (NK) cell lymphocytosis. The patient developed progressive, asymmetric weakness and numbness in all four extremities in the course of a three-month period. Nerve conduction studies revealed asymmetric demyelination in both the motor and sensory nerves, and a biopsy specimen of the sural nerve showed a conspicuous difference in the demyelination between the neighboring fascicles and the infiltration of NK cells in the endoneurium...
September 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28948486/efficacy-and-tolerability-of-phytomedicines-in-multiple-sclerosis-patients-a-review
#18
REVIEW
Mohammad Hosein Farzaei, Zahra Shahpiri, Roodabeh Bahramsoltani, Marjan Moghaddam Nia, Fariba Najafi, Roja Rahimi
Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disorder of the central nervous system (CNS) that can cause cognition, mobility, and sensory impairments. It is considered one of the most common non-traumatic causes of disability in the world. The aim of the present article was to review the clinical evidence related to medicinal plants in the management of MS symptoms. Electronic databases, including the Cochrane Library, Pubmed, and Scopus, were searched for entries from 1966 to February 2017...
September 25, 2017: CNS Drugs
https://www.readbyqxmd.com/read/28941527/pediatric-optic-neuritis
#19
Melinda Y Chang, Stacy L Pineles
Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging, lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Pediatric optic neuritis may occur following infection or vaccination, or in association with a systemic demyelinating process such as acute disseminated encephalomyelitis, neuromyelitis optica, or multiple sclerosis...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28939905/expression-and-differential-responsiveness-of-central-nervous-system-glial-cell-populations-to-the-acute-phase-protein-serum-amyloid-a
#20
Massimo Barbierato, Mila Borri, Laura Facci, Morena Zusso, Stephen D Skaper, Pietro Giusti
Acute-phase response is a systemic reaction to environmental/inflammatory insults and involves hepatic production of acute-phase proteins, including serum amyloid A (SAA). Extrahepatically, SAA immunoreactivity is found in axonal myelin sheaths of cortex in Alzheimer's disease and multiple sclerosis (MS), although its cellular origin is unclear. We examined the responses of cultured rat cortical astrocytes, microglia and oligodendrocyte precursor cells (OPCs) to master pro-inflammatory cytokine tumour necrosis factor (TNF)-α and lipopolysaccaride (LPS)...
September 22, 2017: Scientific Reports
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