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https://www.readbyqxmd.com/read/28527029/critical-flicker-fusion-frequency-in-demyelinating-and-ischemic-optic-neuropathies
#1
Matthew T Young, Puneet S Braich, Scott R Haines
PURPOSE: Critical flicker fusion (CFF) frequency is a well-studied test for evaluating demyelinating optic neuritis (ON). Its use in evaluating other optic neuropathies is not well established. The purpose of this study was to compare CFF in ON and non-arteritic anterior ischemic optic neuropathy (NAION). METHODS: We performed a retrospective review to compare multiple variables for eyes with ON or NAION using two-sided T tests and Chi-square tests. A multivariate linear regression was performed for the dependent variable CFF...
May 19, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28526178/late-onset-progressive-multifocal-leukoencephalopathy-in-hodgkin-lymphoma
#2
Whitley W Aamodt, James E Siegler, Angela N Viaene, Michael N Rubenstein
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease resulting from infection of oligodendrocytes in the central nervous system with John Cunningham virus. Although PML is commonly diagnosed in immunocompromised patients with human immunodeficiency virus, it can also arise in other immunodeficient states. In this report, we present an unusual case of PML occurring 40years after chemoradiation therapy for Hodgkin lymphoma in a patient with normal total lymphocyte counts on annual surveillance...
May 16, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28523213/brachial-plexus-magnetic-resonance-imaging-differentiates-between-inflammatory-neuropathies-and-does-not-predict-disease-course
#3
Bas A Jongbloed, Jeroen W Bos, Dirk Rutgers, Willem Ludo van der Pol, Leonard H van den Berg
OBJECTIVE: The main objective of this study was to evaluate the correlation between the distribution of brachial plexus magnetic resonance imaging (MRI) abnormalities and clinical weakness, and to evaluate the value of brachial plexus MRI in predicting disease course and response to treatment in multifocal motor neuropathy (MMN), Lewis-Sumner syndrome (LSS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: Sixty-seven patients with an inflammatory neuropathy diagnosed at our tertiary referral center for neuromuscular diseases had undergone bilateral T2-weighted short tau inversion recovery (STIR) MRI of the brachial plexus...
May 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28515562/leukodystrophy-presenting-as-hyperactivity-and-bipolarity-with-uncommon-adverse-drug-reaction
#4
Roshan Sutar, Anirban Ray, Shekhar P Sheshadri
Leukodystrophy is a group of demyelinating neurodegenerative diseases of brain with varied presentation and multiple etiologies. Prognosis is predominantly dismal. Misdiagnosis and wrong treatment are common in this group of rare neurological disorders, especially when it presents with psychiatric symptoms. In this case, importance of neurological and radiological evaluation and need for high diagnostic suspicion in treatment-resistant psychiatric disorders is highlighted.
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28515278/critical-role-of-p2y12-receptor-in-regulation-of-th17-differentiation-and-experimental-autoimmune-encephalomyelitis-pathogenesis
#5
Chaoyan Qin, Jinfeng Zhou, Yuan Gao, Weiming Lai, Cuixia Yang, Yingying Cai, Shuai Chen, Changsheng Du
Adenosine 5'-diphosphate is a key endogenous cell-signaling molecule that can activate P2 purinergic receptor family members. ADP-P2Y signaling is reported to be associated with inflammation, but its function in T cell differentiation and autoimmune diseases pathogenesis is unclear. In this study, we found that the P2Y12 receptor was upregulated in the peripheral immune tissues of experimental autoimmune encephalomyelitis (EAE) mice. Deficiency of P2Y12 led to a reduced peak severity and cumulative disease score in EAE mice, followed by a dramatic reduction of leukocyte infiltration and less extensive demyelination...
May 17, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28509084/whole-exome-sequencing-identifies-a-novel-homozygous-frameshift-mutation-in-the-mtmr2-gene-as-a-causative-mutation-in-a-patient-with-charcot-marie-tooth-disease-type-4b1
#6
Tameemi Abdalla-Moady, Amir Peleg, Orit Sadeh, Khader Badarneh, Fuad Fares
Charcot-Marie-Tooth (CMT) disease refers to a heterogeneous group of axonal and demyelinating polyneuropathies, characterized by chronic motor and sensory dysfunction. CMT is the most common genetic cause of neuropathy. The present study aimed to identify the gene mutation responsible for CMT in Ashkenazi Jew (AJ) patient. Genomic DNA was extracted from whole blood leukocytes of affected family and normal subject. Whole-exome sequencing was performed using the Illumina HiSeq2500. The DNA region containing the identified mutation was amplified by PCR and sequenced using dye-terminator chemistry and the forward primer...
May 16, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28507026/lineage-specific-metabolic-properties-and-vulnerabilities-of-t-cells-in-the-demyelinating-central-nervous-system
#7
Scott M Seki, Max Stevenson, Abagail M Rosen, Sanja Arandjelovic, Lelisa Gemta, Timothy N J Bullock, Alban Gaultier
Multiple sclerosis (MS) is a disease that is characterized by immune-mediated destruction of CNS myelin. Current MS therapies aim to block peripheral immune cells from entering the CNS. Although these treatments limit new inflammatory activity in the CNS, no treatment effectively prevents long-term disease progression and disability accumulation in MS patients. One explanation for this paradox is that current therapies are ineffective at targeting immune responses already present in the CNS. To this end, we sought to understand the metabolic properties of T cells that mediate ongoing inflammation in the demyelinating CNS...
May 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28506533/schwann-cell-precursors-from-human-pluripotent-stem-cells-as-a-potential-therapeutic-target-for-myelin-repair
#8
Han-Seop Kim, Jungwoon Lee, Da Yong Lee, Young-Dae Kim, Jae Yun Kim, Hyung Jin Lim, Sungmin Lim, Yee Sook Cho
Schwann cells play a crucial role in successful nerve repair and regeneration by supporting both axonal growth and myelination. However, the sources of human Schwann cells are limited both for studies of Schwann cell development and biology and for the development of treatments for Schwann cell-associated diseases. Here, we provide a rapid and scalable method to produce self-renewing Schwann cell precursors (SCPs) from human pluripotent stem cells (hPSCs), using combined sequential treatment with inhibitors of the TGF-β and GSK-3 signaling pathways, and with neuregulin-1 for 18 days under chemically defined conditions...
May 11, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28501971/electrolyte-and-acid-base-disturbances-in-end-stage-liver-disease-a-physiopathological-approach
#9
REVIEW
José Víctor Jiménez, Diego Luis Carrillo-Pérez, Rodrigo Rosado-Canto, Ignacio García-Juárez, Aldo Torre, David Kershenobich, Eduardo Carrillo-Maravilla
Electrolyte and acid-base disturbances are frequent in patients with end-stage liver disease; the underlying physiopathological mechanisms are often complex and represent a diagnostic and therapeutic challenge to the physician. Usually, these disorders do not develop in compensated cirrhotic patients, but with the onset of the classic complications of cirrhosis such as ascites, renal failure, spontaneous bacterial peritonitis and variceal bleeding, multiple electrolyte, and acid-base disturbances emerge. Hyponatremia parallels ascites formation and is a well-known trigger of hepatic encephalopathy; its management in this particular population poses a risky challenge due to the high susceptibility of cirrhotic patients to osmotic demyelination...
May 13, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28499934/matrine-promotes-oligodendrocyte-development-in-cns-autoimmunity-through-the-pi3k-akt-signaling-pathway
#10
Shuqing Liu, Mingliang Zhang, Huijun Zhang, Fangzhou Liu, Raojuan Chu, Guang-Xian Zhang, Lin Zhu
AIMS: Matrine (MAT), a quinolizidine alkaloid derived from the herb Radix Sophorae flavescens, has been recently found to be beneficial in experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis, mainly through its anti-inflammatory effect. In the present study, we tested the effect of MAT on ongoing EAE and defined possible mechanisms underlying its effects on myelination and oligodendrocytes. MAIN METHODS: EAE was induced in C57BL/6 mice and MAT treatment was started at disease onset...
May 9, 2017: Life Sciences
https://www.readbyqxmd.com/read/28495143/central-and-peripheral-nervous-system-immune-mediated-demyelinating-disease-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
M I Stefanou, F Bischof
OBJECTIVE: We aimed to evaluate clinical and diagnostic features of central and peripheral immune-mediated demyelinating disease (CPID) in allogeneic hematopoietic stem cell transplantation (aHSCT) recipients. BACKGROUND: CPID refers to the late-onset, immune-mediated neurological complications following aHSCT, when other frequent differential diagnoses have been ruled out, and when symptoms and signs of systemic GvHD manifestations are absent. METHODS: Case records at the University of Tuebingen, between 2001 and 2015, were screened to identify patients with CPID after aHSCT...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495131/massive-intracerebral-epstein-barr-virus-reactivation-in-lethal-multiple-sclerosis-relapse-after-natalizumab-withdrawal
#12
Barbara Serafini, Eleonora Scorsi, Barbara Rosicarelli, Valérie Rigau, Eric Thouvenot, Francesca Aloisi
Rebound of disease activity in multiple sclerosis patients after natalizumab withdrawal is a potentially life-threatening event. To verify whether highly destructive inflammation after natalizumab withdrawal is associated with Epstein-Barr virus (EBV) reactivation in central nervous system infiltrating B-lineage cells and cytotoxic immunity, we analyzed post-mortem brain tissue from a patient who died during a fulminating MS relapse following natalizumab withdrawal. Numerous EBV infected B cells/plasma cells and CD8+ T cells infiltrated all white matter lesions; the highest frequency of EBV lytically infected cells and granzyme B+ CD8+ T cells was observed in actively demyelinating lesions...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495047/similar-clinical-pathological-and-genetic-features-in-chinese-patients-with-autosomal-recessive-and-dominant-charcot-marie-tooth-disease-type-2k
#13
Jun Fu, Shixu Dai, Yuanyuan Lu, Rui Wu, Zhaoxia Wang, Yun Yuan, He Lv
Mutations in the ganglioside-induced differentiation-associated protein 1 gene (GDAP1) cause rare subtypes of Charcot-Marie-Tooth disease (CMT2K and CMT4A). CMT2K is an axonal neuropathy while CMT4A is a demyelinating type. In a series of 169 Chinese CMT patients (79 CMT1, 52 CMT2 and 38 unclassified), four unrelated patients (2.37%) were identified with GDAP1 mutations, including two with autosomal recessive CMT2K (AR-CMT2K) and two dominant CMT2K (AD-CMT2K). All patients had disease onset before 5 years of age, and presented with muscle weakness, atrophy, and mild sensory disturbance in distal limbs...
April 20, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28493815/the-biology-of-jc-polyomavirus
#14
Benedetta Assetta, Walter J Atwood
JC polyomavirus (JCPyV) is the causative agent of a fatal central nervous system demyelinating disease known as progressive multifocal leukoencephalopathy (PML). PML occurs in people with underlying immunodeficiency or in individuals being treated with potent immunomodulatory therapies. JCPyV is a DNA tumor virus with a double stranded DNA genome and encodes a well-studied oncogene, large T antigen. Its host range is highly restricted to humans and only a few cell types support lytic infection in vivo or in vitro...
May 10, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/28493141/therapeutic-strategies-in-adrenoleukodystrophy
#15
Bela R Turk, Ann B Moser, Ali Fatemi
Adrenoleukodystrophy (ALD) is an X‑linked hereditary disorder due to mutations of the ABCD1 gene, which encodes a peroxisomal transport protein necessary for very long-chain fatty acid degradation (VLCFA). Toxic accumulation thereof is associated with a proinflammatory state and eventual cell death in multiple tissues. ALD may manifest either as a fatal, rapidly progressive demyelinating disease in boys and adult men, or as a slowly progressive adult-onset long-tract myelopathy along with peripheral neuropathy...
May 10, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28489810/a-case-report-of-hereditary-neuropathy-with-liability-to-pressure-palsies-accompanied-by-type-2-diabetes-mellitus-and-psoriasis
#16
Jing Li, Bing Niu, Xiaoling Wang, Huaiqiang Hu, Bingzhen Cao
RATIONALE: Hereditary neuropathy with liability to pressure palsy (HNPP) is an episodic, multifocal neuropathy, with a typical clinical presentation of recurrent transient pressure palsies, which is induced by a PMP22 deletion. Another neuropathy caused by a PMP22 duplication is Charcot-Marie-Tooth disease type 1A (CMT1A). PMP22 is a gene coding a protein called peripheral myelin protein 22 (PMP22), which plays an essential role in the formation and maintenance of compact myelin. Coexistence of type 2 diabetes mellitus (T2DM) and CMT1A has been reported in many work, however HNPP patients with T2DM are rare, and comorbidity of HNPP and psoriasis has not been reported previously...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28488621/tumefactive-acute-disseminated-encephalomyelitis
#17
Sunil Pradhan, Surjyaprakash S Choudhury, Animesh Das
Tumefactive demyelinating lesions are tumour-like presentations of acute demyelinating lesions. They have been described with multiple sclerosis only and not with other varieties of acquired demyelination like acute disseminated encephalomyelitis (ADEM). The uncertainty about the diagnosis at the onset of the disease in tumefactive ADEM makes it important that the physicians should be aware of this entity. Various radiological similarities with more sinister lesions like central nervous system gliomas or lymphomas may lead to this confusion...
May 2017: Neurology India
https://www.readbyqxmd.com/read/28485884/altered-sensorimotor-cortical-oscillations-in-individuals-with-multiple-sclerosis-suggests-a-faulty-internal-model
#18
David J Arpin, Elizabeth Heinrichs-Graham, James E Gehringer, Rana Zabad, Tony W Wilson, Max J Kurz
Multiple sclerosis (MS) is a demyelinating disease that results in a broad array of symptoms, including impaired motor performance. How such demyelination of fibers affects the inherent neurophysiological activity in motor circuits, however, remains largely unknown. Potentially, the movement errors associated with MS may be due to imperfections in the internal model used to make predictions of the motor output that will meet the task demands. Prior magnetoencephalographic (MEG) and electroencephalographic brain imaging experiments have established that the beta (15-30 Hz) oscillatory activity in the sensorimotor cortices is related to the control of movement...
May 9, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28484224/suppression-of-autoimmune-demyelinating-disease-by-preferential-stimulation-of-cns-specific-cd8-t-cells-using-listeria-encoded-neuroantigen
#19
Farah R Itani, Sushmita Sinha, Ashley A Brate, Lecia L Pewe, Katherine N Gibson-Corley, John T Harty, Nitin J Karandikar
CD8 T-cells predominate in CNS lesions of MS patients and display oligoclonal expansion. However, the role of myelin-specific CD8 T-cells in disease remains unclear, with studies showing protective and pathogenic roles in EAE. We demonstrated a disease-suppressive function for CNS-specific CD8 T-cells in a model where the antigen is exogenously administered in vivo and used for in vitro activation. To probe the nature of the CD8 response elicited by endogenously presented myelin antigens in vivo, we developed a novel approach utilizing infection with Listeria monocytogenes (LM) encoding proteolipid protein peptide (PLP) amino acids 178-191 (LM-PLP)...
May 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28481421/immunomodulatory-treatment-other-than-corticosteroids-immunoglobulin-and-plasma-exchange-for-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#20
REVIEW
Mohamed Mahdi-Rogers, Ruth Brassington, Angela A Gunn, Pieter A van Doorn, Richard Ac Hughes
BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disease that causes progressive or relapsing and remitting weakness and numbness. It is probably caused by an autoimmune process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. This review was first published in 2003 and has been updated most recently in 2016. OBJECTIVES: To assess the effects of immunomodulatory and immunosuppressive agents other than corticosteroids, immunoglobulin, and plasma exchange in CIDP...
May 8, 2017: Cochrane Database of Systematic Reviews
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