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https://www.readbyqxmd.com/read/27930572/acute-onset-chronic-inflammatory-demyelinating-polyneuropathy-in-hantavirus-and-hepatitis-b-virus-coinfection-a-case-report
#1
Jong Youb Lim, Young-Ho Lim, Eun-Hi Choi
INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disorder with progressive weakness. Acute-onset CIDP resembles Guillain-Barre syndrome (GBS), a rapidly progressive disorder, and follows a chronic course. To our knowledge, no case of acute-onset CIDP in hantavirus and hepatitis B virus (HBV) coinfection has been reported previously. CLINICAL FINDINGS: We report a case of acute-onset CIDP that was initially diagnosed as GBS...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27930320/unexpected-central-role-of-the-androgen-receptor-in-the-spontaneous-regeneration-of-myelin
#2
Bartosz Bielecki, Claudia Mattern, Abdel M Ghoumari, Sumaira Javaid, Kaja Smietanka, Charly Abi Ghanem, Sakina Mhaouty-Kodja, M Said Ghandour, Etienne-Emile Baulieu, Robin J M Franklin, Michael Schumacher, Elisabeth Traiffort
Lost myelin can be replaced after injury or during demyelinating diseases in a regenerative process called remyelination. In the central nervous system (CNS), the myelin sheaths, which protect axons and allow the fast propagation of electrical impulses, are produced by oligodendrocytes. The abundance and widespread distribution of oligodendrocyte progenitors (OPs) within the adult CNS account for this remarkable regenerative potential. Here, we report a key role for the male gonad, testosterone, and androgen receptor (AR) in CNS remyelination...
December 7, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27927941/a-population-based-epidemiologic-study-of-adult-neuromuscular-disease-in-the-republic-of-ireland
#3
Stela Lefter, Orla Hardiman, Aisling M Ryan
OBJECTIVE: To estimate the prevalence rates (PRs) of acquired and inherited neuromuscular diseases (NMD) in the adult Irish population, reflecting the burden of these conditions in a single country. METHODS: This population-based study was performed in the Republic of Ireland (RoI), with a PR estimated for December 2013. Multiple case ascertainment sources were utilized. Demographic and clinical information and relevant diagnostic results were registered. RESULTS: A total of 2,641 adults were identified, giving a PR of 62...
December 7, 2016: Neurology
https://www.readbyqxmd.com/read/27920424/ultrastructural-characterization-of-the-lower-motor-system-in-a-mouse-model-of-krabbe-disease
#4
Valentina Cappello, Laura Marchetti, Paola Parlanti, Silvia Landi, Ilaria Tonazzini, Marco Cecchini, Vincenzo Piazza, Mauro Gemmi
Krabbe disease (KD) is a neurodegenerative disorder caused by the lack of β- galactosylceramidase enzymatic activity and by widespread accumulation of the cytotoxic galactosyl-sphingosine in neuronal, myelinating and endothelial cells. Despite the wide use of Twitcher mice as experimental model for KD, the ultrastructure of this model is partial and mainly addressing peripheral nerves. More details are requested to elucidate the basis of the motor defects, which are the first to appear during KD onset. Here we use transmission electron microscopy (TEM) to focus on the alterations produced by KD in the lower motor system at postnatal day 15 (P15), a nearly asymptomatic stage, and in the juvenile P30 mouse...
December 2016: Scientific Reports
https://www.readbyqxmd.com/read/27904823/expression-of-gp91phox-and-p22phox-catalytic-subunits-of-nadph-oxidase-on-microglia-in-nasu-hakola-disease-brains
#5
Jun-Ichi Satoh, Yoshihiro Kino, Motoaki Yanaizu, Youhei Tosaki, Kenji Sakai, Tusyoshi Ishida, Yuko Saito
The superoxide-producing nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytes (phox) plays a key role in production of reactive oxygen species (ROS) by microglia. The catalytic subunits of the NADPH oxidase are composed of p22phox and gp91phox. Nasu-Hakola disease (NHD) is a rare autosomal recessive disorder caused by a loss-of-function mutation of either TYROBP (DAP12) or TREM2. Pathologically, the brains of NHD patients exhibit extensive demyelination designated leukoencephalopathy, astrogliosis, accumulation of axonal spheroids, and remarkable activation of microglia predominantly in the white matter of frontal and temporal lobes...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27902997/chronic-inflammatory-demyelinating-polyradiculoneuropathy-cidp-clinical-features-diagnosis-and-current-treatment-strategies
#6
Jacques Reynolds, George Sachs, Kara Stavros
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits that can lead to significant neurological disability. The diagnosis is based on a combination of clinical examination findings, electrodiagnostic studies, and other supportive evidence. Recognizing CIDP and distinguishing it from other chronic polyneuropathies is important because many patients with CIDP are highly responsive to treatment with immunosuppressive or immunomodulatory therapies...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27900773/systematic-review-of-immunoglobulin-use-in-paediatric-neurological-and-neurodevelopmental-disorders
#7
REVIEW
Jonathan Gadian, Emma Kirk, Kate Holliday, Ming Lim, Michael Absoud
AIM: A systematic literature review of intravenous immunoglobulin (IVIG) treatment of paediatric neurological conditions was performed to summarize the evidence, provide recommendations, and suggest future research. METHOD: A MEDLINE search for articles reporting on IVIG treatment of paediatric neuroinflammatory, neurodevelopmental, and neurodegenerative conditions published before September 2015, excluding single case reports and those not in English. Owing to heterogeneous outcome measures, meta-analysis was not possible...
November 30, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27894447/differentiating-familial-neuropathies-from-guillain-barr%C3%A3-syndrome
#8
REVIEW
Brett J Bordini, Priya Monrad
Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27889190/diagnosis-of-multiple-sclerosis-progress-and-challenges
#9
REVIEW
Wallace J Brownlee, Todd A Hardy, Franz Fazekas, David H Miller
The diagnosis of multiple sclerosis is based on neurological symptoms and signs, alongside evidence of dissemination of CNS lesions in space and time. MRI is often sufficient to confirm the diagnosis when characteristic lesions accompany a typical clinical syndrome, but in some patients, further supportive information is obtained from cerebrospinal fluid examination and neurophysiological testing. Differentiation is important from other diseases in which demyelination is a feature (eg, neuromyelitis optica spectrum disorder and acute disseminated encephalomyelitis) and from non-demyelinating disorders such as chronic small vessel disease and other inflammatory, granulomatous, infective, metabolic, and genetic causes that can mimic multiple sclerosis...
November 23, 2016: Lancet
https://www.readbyqxmd.com/read/27883356/a-human-brain-microphysiological-system-derived-from-induced-pluripotent-stem-cells-to-study-neurological-diseases-and-toxicity
#10
David Pamies, Paula Barreras, Katharina Block, Georgia Makri, Anupama Kumar, Daphne Wiersma, Lenna Smirnova, Ce Zhang, Joseph Bressler, Kimberly M Christian, Georgina Harris, Guo-Li Ming, Cindy J Berlinicke, Kelly Kyro, Hongjun Song, Carlos A Pardo, Thomas Hartung, Helena T Hogberg
Human in-vitro models of brain neurophysiology are needed to investigate molecular and cellular mechanisms associated with neurological disorders and neurotoxicity. We have developed a reproducible iPSC-derived human 3D brain microphysiological system (BMPS), comprised of differentiated mature neurons and glial cells (astrocytes and oligodendrocytes) that reproduce neuronal-glial interactions and connectivity. BMPS mature over eight weeks and show the critical elements of neuronal function: synaptogenesis and neuron-to-neuron (e...
November 24, 2016: ALTEX
https://www.readbyqxmd.com/read/27882183/psychiatric-symptoms-and-limb-tremors-associated-with-central-pontine-myelinolysis-a-case-of-alcoholism-without-hyponatremia
#11
Xue-Min Feng, Teng Zhao, Chun-Kui Zhou, Jing-Yao Liu
Central pontine myelinolysis (CPM), also known as osmotic demyelination syndrome, is a rare demyelinating disorder characterized by the loss of myelin in the center of the basis pontis. In this case report, an alcoholic patient with CPM and acquired demyelinating lesion of the basis pontis is described. The patient is a 70 year-old woman who presented with intermittent psychiatric symptoms and limb tremors following two months of alcohol abuse. During admission, magnetic resonance imaging (MRI) revealed hyperintensity on T2 weighted images and fluid-attenuated inversion-recovery imaging in the central pons without contrast enhancement...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27874947/the-relationship-between-the-occurrence-of-intractable-epilepsy-with-glial-cells-and-myelin-sheath-an-experimental-study
#12
X Hu, J-Y Wang, R Gu, H Qu, M Li, L Chen, R Liu, P Yuan
OBJECTIVE: The occurrence of epilepsy is associated with myelin sheath injury; oligodendrocyte (OL) is the main cell of myelin sheath; In this study, we observed the changes of OL, demyelination, and myelin associated protein in different stages of intractable epilepsy (IE) at the epileptic foci of patients, and provide useful information for the pathophysiology of IE. PATIENTS AND METHODS: IE patients who received epileptogenic focus resection were recruited as the experimental group, their medical records were collected and postoperative follow-up was performed...
November 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27872470/hereditary-amyloidosis-with-recurrent-lung-infiltrates
#13
Alberto E Revelo, Crischelle Magaspi, George Maguire, Wilbert S Aronow
BACKGROUND Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement...
November 22, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27872169/myelopathy-chameleons-and-mimics
#14
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...
November 21, 2016: Practical Neurology
https://www.readbyqxmd.com/read/27864848/age-related-small-vessel-disease-a-potential-contributor-to-neurodegeneration-in-multiple-sclerosis
#15
REVIEW
Ruth Geraldes, Margaret M Esiri, Gabriele C DeLuca, Jacqueline Palace
Multiple sclerosis (MS) is a chronic inflammatory demyelinating disorder of the central nervous system wherein, after an initial phase of transient neurological defects, slow neurological deterioration due to progressive neuronal loss ensues. Age is a major determinant of MS progression onset and disability. Over the past years, several mechanisms have been proposed to explain the key drivers of neurodegeneration and disability accumulation in MS. However, the effect of commonly encountered age-related cerebral vessel disease, namely small vessel disease (SVD), has been largely neglected and constitutes the aim of this review...
November 19, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27861905/attenuation-of-experimental-autoimmune-neuritis-with-locally-administered-lovastatin-encapsulating-plga-nanoparticles
#16
Kelly A Langert, Bruktawit Goshu, Evan B Stubbs
Acute inflammatory demyelinating polyneuropathy (AIDP) is an aggressive antibody- and T cell-mediated variant of Guillain-Barré Syndrome (GBS), a prominent and debilitating autoimmune disorder of the peripheral nervous system. Despite advancements in clinical management, treatment of patients with AIDP/GBS and its chronic variant CIDP remains palliative and relies on the use of non-specific immune-modulating therapies. Our laboratory has previously reported that therapeutic administration of statins safely attenuates the clinical severity of experimental autoimmune neuritis (EAN), a well-characterized animal model of AIDP/GBS, by restricting the migration of autoreactive leukocytes across peripheral nerve microvascular endoneurial endothelial cells (PNMECs) that form the blood-nerve barrier...
November 8, 2016: Journal of Neurochemistry
https://www.readbyqxmd.com/read/27857121/guillain-barr%C3%A3-syndrome-a-century-of-progress
#17
REVIEW
John A Goodfellow, Hugh J Willison
In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts - novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise...
November 18, 2016: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/27855220/disease-type-and-status-specific-alteration-of-csf-metabolome-coordinated-with-clinical-parameters-in-inflammatory-demyelinating-diseases-of-cns
#18
Soo Jin Park, In Hye Jeong, Byung Soo Kong, Jung-Eun Lee, Kyoung Heon Kim, Do Yup Lee, Ho Jin Kim
Central nervous system (CNS) inflammatory demyelinating diseases (IDDs) are a group of disorders with different aetiologies, characterized by inflammatory lesions. These disorders include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and idiopathic transverse myelitis (ITM). Differential diagnosis of the CNS IDDs still remains challenging due to frequent overlap of clinical and radiological manifestation, leading to increased demands for new biomarker discovery. Since cerebrospinal fluid (CSF) metabolites may reflect the status of CNS tissues and provide an interfacial linkage between blood and CNS tissues, we explored multi-component biomarker for different IDDs from CSF samples using gas chromatography mass spectrometry-based metabolite profiling coupled to multiplex bioinformatics approach...
2016: PloS One
https://www.readbyqxmd.com/read/27854160/lamin-b1-mediated-demyelination-linking-lamins-lipids-and-leukodystrophies
#19
Quasar S Padiath
Autosomal Dominant Leukodystrophy (ADLD), a fatal adult onset demyelinating disorder, is the only human disease that has been linked to mutations of the nuclear lamina protein, lamin B1, and is primarily caused by duplications of the LMNB1 gene. Why CNS myelin is specifically targeted and the mechanisms underlying ADLD are unclear. Recent work from our group has demonstrated that over expression of lamin B1 in oligodendrocytes, the myelin producing cells in the CNS, resulted in age dependent epigenetic modifications, transcriptional down-regulation of lipogenic gene expression and significant reductions of myelin-enriched lipids...
November 17, 2016: Nucleus
https://www.readbyqxmd.com/read/27853448/a-novel-mutation-in-abca1-gene-causing-tangier-disease-in-an-italian-family-with-uncommon-neurological-presentation
#20
Marco Ceccanti, Chiara Cambieri, Vittorio Frasca, Emanuela Onesti, Antonella Biasiotta, Carla Giordano, Sabina M Bruno, Giancarlo Testino, Marco Lucarelli, Marcello Arca, Maurizio Inghilleri
Tangier disease is an autosomal recessive disorder characterized by severe reduction in high-density lipoprotein cholesterol and peripheral lipid storage. We describe a family with c.5094C > A p.Tyr1698* mutation in the ABCA1 gene, clinically characterized by syringomyelic-like anesthesia, demyelinating multineuropathy, and reduction in intraepidermal small fibers innervation. In the proband patient, cardiac involvement determined a myocardial infarction; lipid storage was demonstrated in gut, cornea, and aortic wall...
2016: Frontiers in Neurology
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