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https://www.readbyqxmd.com/read/27931119/assessing-cognitive-functioning-in-als-a-focus-on-frontal-lobe-processes
#1
S M Gillingham, Y Yunusova, A Ganda, E Rogaeva, S E Black, D T Stuss, L Zinman
OBJECTIVE: It is generally acknowledged that at least 50% of individuals with amyotrophic lateral sclerosis (ALS) will exhibit cognitive deficits outside of the characteristic motor neuron involvement. However, a specific cognitive profile has been difficult to ascertain due to disease-related testing barriers and limitations in the sensitivity and specificity of available assessment methods. This study assessed the level of functioning of extramotor frontal cognitive processes in ALS, and the amount of change in the functioning in these processes over time as disease progresses...
December 8, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27920682/scleroderma-with-nodular-scleroderma
#2
Chutika Srisuttiyakorn, Kobkul Aunhachoke
BACKGROUND: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. MAIN OBSERVATIONS: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27920668/getting-mirna-therapeutics-into-the-target-cells-for-neurodegenerative-diseases-a-mini-review
#3
REVIEW
Ming Ming Wen
miRNAs play important roles in modulating gene expression in varying cellular processes and disease pathogenesis, including neurodegenerative diseases. Several miRNAs are expressed in the brain, control brain development and are identified as important biomarkers in the pathogenesis of motor-and neuro-cognitive diseases such as Alzheimer's (AD), Huntington's and Parkinson's diseases (PD) and amyotrophic lateral sclerosis. These remarkable miRNAs could be used as diagnostic markers and therapeutic targeting potential for many stressful and untreatable progressive neurodegenerative diseases...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27920149/mir-142-3p-is-a-key-regulator-of-il-1%C3%AE-dependent-synaptopathy-in-neuroinflammation
#4
Georgia Mandolesi, Francesca De Vito, Alessandra Musella, Antonietta Gentile, Silvia Bullitta, Diego Fresegna, Helena Sepman, Claudio Di Sanza, Nabila Haji, Francesco Mori, Fabio Buttari, Emerald Perlas, Maria Teresa Ciotti, Eran Hornstein, Irene Bozzoni, Carlo Presutti, Diego Centonze
: MicroRNAs (miRNA) play an important role in posttranscriptional gene regulation of several physiological and pathological processes. In multiple sclerosis (MS), a chronic inflammatory and degenerative disease of the CNS, and in its mouse model, the experimental autoimmune encephalomyelitis (EAE), miRNA dysregulation has been mainly related to immune system dysfunction and white matter pathology. However, little is known about their role in grey matter pathology. Here, we explored miRNA involvement in the inflammation-driven alterations of synaptic structure and function, collectively known as synaptopathy, a neuropathological process contributing to excitotoxic neurodegeneration in MS/EAE...
December 5, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27918067/systematic-approach-to-understanding-the-pathogenesis-of-systemic-sclerosis
#5
REVIEW
Xiao Xia Zuo, Li Hua Zhang, Hui Luo, Yisha Li, Honglin Zhu
Systemic sclerosis (SSc) is a complex heterogeneous autoimmune disease. Progressive organ fibrosis is a major contributor to SSc mortality. Despite extensive efforts, the underlying mechanism of SSc remains unclear. Efforts to understand the pathogenesis of SSc have included genomics, epigenetics, transcriptomic, proteomic and metabolomic studies in the last decade. This review focuses on recent studies in SSc research based on multi-omics. The combination of these technologies can help us understand the pathogenesis of SSc...
December 5, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27914464/immunogenicity-of-human-interferon-beta-containing-pharmaceuticals
#6
V D Nazarov, S V Lapin, A V Mazing, E P Evdoshenko, A A Totolian
Multiple sclerosis is a severe autoimmune disease with inflammatory component that continues to be resistant to treatment. One of the approaches retarding its progression is based on using nonspecific therapy with human interferon-beta (IFN-β)-containing pharmaceuticals. Neutralizing antibodies (NAbs) against genetically engineered pharmaceuticals developed by the patient's immune system, which reduce their therapeutic and biological activity, pose a serious problem. Cell lines sensitive to IFN-β activity also quantifying NAb level are applied because direct measurement of IFN-β antiviral activity is complicated...
November 2016: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/27911409/measuring-progressive-neurological-disability-in-a-mouse-model-of-multiple-sclerosis
#7
Francesca Gilli, Darlene B Royce, Andrew R Pachner
After intracerebral infection with the Theiler's Murine Encephalomyelitis Virus (TMEV), susceptible SJL mice develop a chronic-progressive demyelinating disease, with clinical features similar to the progressive forms of multiple sclerosis (MS). The mice show progressive disability with loss of motor and sensory functions, which can be assessed with multiple apparatuses and protocols. Among them, the Rotarod performance test is a very common behavioral test, its advantage being that it provides objective measurements, but it is often used assuming that it is straightforward and simple...
November 14, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27908301/utility-of-b-type-natriuretic-peptides-in-the-assessment-of-patients-with-systemic-sclerosis-associated-pulmonary-hypertension-in-the-pharos-registry
#8
Lorinda Chung, Robert M Fairchild, Daniel E Furst, Shufeng Li, Firas Alkassab, Marcy B Bolster, Mary Ellen Csuka, Chris T Derk, Robyn T Domsic, Aryeh Fischer, Tracy Frech, Mardi Gomberg-Maitland, Jessica K Gordon, Monique Hinchcliff, Vivien Hsu, Laura K Hummers, Dinesh Khanna, Thomas A Jr Medsger, Jerry A Molitor, Ivana R Preston, Elena Schiopu, Lee Shapiro, Faye Hant, Richard Silver, Robert Simms, John Varga, Virginia D Steen, Roham T Zamanian
OBJECTIVES: To assess the utility of B-type natriuretic peptide (BNP) and N-terminal pro-BNP (NT-proBNP) in detecting and monitoring pulmonary hypertension (PH) in systemic sclerosis (SSc). METHODS: PHAROS is a multicenter prospective cohort of SSc patients at high risk for developing pulmonary arterial hypertension (SSc-AR-PAH) or with a definitive diagnosis of SSc-PH. We evaluated 1) the sensitivity and specificity of BNP≥64 and NT-proBNP≥210 pg/mL for the detection of SSc-PAH and/ or SSc-PH in the SSc-AR-PAH population; 2) baseline and longitudinal BNP and NT-proBNP levels as predictors of progression to SSc-PAH and/or SSc-PH; 3) baseline BNP≥180, NT-proBNP≥553 pg/mL, and longitudinal changes in BNP and NT-proBNP as predictors of mortality in SSc-PH diagnosed patients...
November 10, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27908171/automated-detection-of-white-matter-hyperintensities-of-all-sizes-in-cerebral-small-vessel-disease
#9
Mohsen Ghafoorian, Nico Karssemeijer, Inge W M van Uden, Frank-Erik de Leeuw, Tom Heskes, Elena Marchiori, Bram Platel
PURPOSE: White matter hyperintensities (WMH) are seen on FLAIR-MRI in several neurological disorders, including multiple sclerosis, dementia, Parkinsonism, stroke and cerebral small vessel disease (SVD). WMHs are often used as biomarkers for prognosis or disease progression in these diseases, and additionally longitudinal quantification of WMHs is used to evaluate therapeutic strategies. Human readers show considerable disagreement and inconsistency on detection of small lesions. A multitude of automated detection algorithms for WMHs exists, but since most of the current automated approaches are tuned to optimize segmentation performance according to Jaccard or Dice scores, smaller WMHs often go undetected in these approaches...
December 2016: Medical Physics
https://www.readbyqxmd.com/read/27906103/progressive-impairment-of-cav1-1-function-in-the-skeletal-muscle-of-mice-expressing-a-mutant-type-1-cu-zn-superoxide-dismutase-g93a-linked-to-amyotrophic-lateral-sclerosis
#10
Donald Beqollari, Christin F Romberg, Gabriella Dobrowolny, Martina Martini, Andrew A Voss, Antonio Musarò, Roger A Bannister
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that is typically fatal within 3-5 years of diagnosis. While motoneuron death is the defining characteristic of ALS, the events that underlie its pathology are not restricted to the nervous system. In this regard, ALS muscle atrophies and weakens significantly before presentation of neurological symptoms. Since the skeletal muscle L-type Ca(2+) channel (CaV1.1) is a key regulator of both mass and force, we investigated whether CaV1...
June 23, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27903258/catwalk-gait-analysis-in-a-rat-model-of-multiple-sclerosis
#11
Sabine Herold, Prateek Kumar, Klaus Jung, Irina Graf, Henrike Menkhoff, Xenia Schulz, Mathias Bähr, Katharina Hein
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-induced experimental autoimmune encephalomyelitis (EAE) is a widely used animal model for multiple sclerosis. The characteristic feature of the MOG-EAE model in Brown Norway rats is consistent involvement of the spinal cord resulting in limb paresis. The aim of the study was to investigate whether early subclinical gait abnormalities are present in this animal model and can be detected by CatWalk XT, a fully automated gait analysis system...
November 30, 2016: BMC Neuroscience
https://www.readbyqxmd.com/read/27899043/use-of-biologics-and-other-novel-therapies-for-the-treatment-of-systemic-sclerosis
#12
Cosimo Bruni, Emanuela Praino, Yannick Allanore, Oliver Distler, Armando Gabrielli, Florenzo Iannone, Marco Matucci-Cerinic
Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation and fibrosis. These three main disease-determining pathways are the target of the currently available treatments used to possibly modify the progression of disease-related manifestations, although this synergy has not been fully applied on SSc joint, skin or lung involvement yet. Areas covered: we describe the current status of SSc treatment/therapy performing a literature search in MEDLINE/Pubmed and Thomson Reuter's Web of Science for articles published until March 2016...
November 30, 2016: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/27895400/esophagogastric-anastomosis-in-rats-improved-healing-by-bpc-157-and-l-arginine-aggravated-by-l-name
#13
Zeljko Djakovic, Ivka Djakovic, Vedran Cesarec, Goran Madzarac, Tomislav Becejac, Goran Zukanovic, Domagoj Drmic, Lovorka Batelja, Anita Zenko Sever, Danijela Kolenc, Alen Pajtak, Nikica Knez, Mladen Japjec, Kresimir Luetic, Dinko Stancic-Rokotov, Sven Seiwerth, Predrag Sikiric
AIM: To cure typically life-threatening esophagogastric anastomosis in rats, lacking anastomosis healing and sphincter function rescue, in particular. METHODS: Because we assume esophagogastric fistulas represent a particular NO-system disability, we attempt to identify the benefits of anti-ulcer stable gastric pentadecapeptide BPC 157, which was in trials for ulcerative colitis and currently for multiple sclerosis, in rats with esophagocutaneous fistulas. Previously, BPC 157 therapies have promoted the healing of intestinal anastomosis and fistulas, and esophagitis and gastric lesions, along with rescued sphincter function...
November 7, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27894110/significant-role-s-of-cxcl12-and-the-sdf-1-3-a-genetic-variant-in-the-pathogenesis-of-multiple-sclerosis
#14
Mojgan Noroozi Karimabad, Soudeh Khanamani Falahati-Pour, Gholamhossein Hassanshahi
Both cellular and molecular components of the immune system are among the substantial factors involved in the pathogenesis of multiple sclerosis (MS). Accumulating evidence confirms that chemokines, as the main members of the immune system, play key roles in the regulation of immune responses. Immune system genetic parameters are believed to influence the onset of immune system-related diseases. Regarding the significant role of the CXCR4/CXCL12 axis in cell differentiation and survival and homing of hematopoietic progenitors to the bone marrow and regulation of neuronal progenitor cell migration in the central nervous system (CNS), genetic factors can cause an increased expression of CXCL12 and induce a vigorous immune response against CNS antigens in MS patients...
November 29, 2016: Neuroimmunomodulation
https://www.readbyqxmd.com/read/27893883/use-of-magnetic-resonance-imaging-to-visualize-leptomeningeal-inflammation-in-patients-with-multiple-sclerosis-a-review
#15
Jonathan Zurawski, Hans Lassmann, Rohit Bakshi
Importance: Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system traditionally characterized by an initial relapsing-remitting clinical course and focal inflammatory lesions that have a predilection for the periventricular white matter. Recently, however, histopathologic and imaging studies have illustrated a more complex pathologic substrate involving cortical demyelination, gray matter atrophy, and meningeal inflammation. Neuroimaging advances have facilitated improved detection of cortical pathology, but our understanding of the pathogenesis of cortical disease remains incomplete...
November 28, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27892723/optimization-and-stratification-of-multiple-sclerosis-treatment-in-fast-developing-economic-countries-a-perspective-from-qatar
#16
Dirk Deleu, Boulenouar Mesraoua, Hisham El Khider, Beatriz Canibano, Gayane Melikyan, Hassan Al Hail, Noha Mhjob, Anjushri Bhagat, Faiza Ibrahim, Yolande Hanssens
OBJECTIVE: The introduction of disease-modifying therapies (DMTs) - with varying degrees of efficacy for reducing annual relapse rate and disability progression - has considerably transformed the therapeutic landscape of relapsing-remitting multiple sclerosis (RRMS). We aim to develop rational evidence-based treatment recommendations and algorithms for the management of clinically isolated syndrome (CIS) and RRMS conform to the healthcare system in a fast-developing economic country such as Qatar...
November 28, 2016: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/27892702/slowly-progressive-motor-neuron-disease-with-multi-system-involvement-related-to-p-e121g-sod1-mutation
#17
Guillaume Taieb, Anne Polge, Raul Juntas-Morales, Nicolas Pageot, Serge Lumbroso, Kevin Mouzat, William Camu
We report the third case of amyotrophic lateral sclerosis related to p.E121G Superoxide dismutase-1 (SOD1) mutation. Besides a sporadic presentation and a slow progressive course, as described in the 2 previously cases, our patient presented with prominent sensory and cerebellar signs. This case report strengthens that p.E121G should be considered as a causal mutation. Slowly upper and lower motor neuron degeneration, even with non-motor clinical features, should prompt a sequencing of SOD1.
November 28, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27890706/the-sphingosine-1-phosphate-receptor-a-novel-therapeutic-target-for-multiple-sclerosis-and-other-autoimmune-diseases
#18
REVIEW
Yang Mao-Draayer, Jeffrey Sarazin, David Fox, Elena Schiopu
Multiple sclerosis (MS) is a prototype autoimmune disease of the central nervous system (CNS). Currently, there is no drug that provides a cure for MS. To date, all immunotherapeutic drugs target relapsing remitting MS (RR-MS); it remains a daunting medical challenge in MS to develop therapy for secondary progressive MS (SP-MS). Since the approval of the non-selective sphingosine-1-phosphate (S1P) receptor modulator FTY720 (fingolimod [Gilenya®]) for RR-MS in 2010, there have been many emerging studies with various selective S1P receptor modulators in other autoimmune conditions...
November 23, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27889559/plasma-endothelial-microparticles-reflect-the-extent-of-capillaroscopic-alterations-and-correlate-with-the-severity-of-skin-involvement-in-systemic-sclerosis
#19
Małgorzata Michalska-Jakubus, Otylia Kowal-Bielecka, Vanessa Smith, Maurizio Cutolo, Dorota Krasowska
INTRODUCTION AND AIM: Endothelial microparticles (EMPs) are membrane-coated vesicles shed from endothelial cells and are considered markers of the endothelial state. It has been shown that total numbers of circulating EMPs are increased in patients with systemic sclerosis (SSc), but their clinical correlations have not yet been investigated in detail. We aimed to assess possible relationships between circulating EMPs and clinical as well as laboratory features among SSc patients with special attention to possible association with alteration in microvascular morphology objectified on nailfold videocapillaroscopy and clinical signs of microvascular complications...
November 23, 2016: Microvascular Research
https://www.readbyqxmd.com/read/27888187/what-does-the-alsfrs-r-really-measure-a-longitudinal-and-survival-analysis-of-functional-dimension-subscores-in-amyotrophic-lateral-sclerosis
#20
James Rooney, Tom Burke, Alice Vajda, Mark Heverin, Orla Hardiman
INTRODUCTION: ALS functional rating scale (revised) (ALSFRS-R) is the most widely used functional rating system in patients with amyotrophic lateral sclerosis (ALS). However, heterogeneity in ALSFRS-R progression renders analysis challenging. We have explored the characteristics of total ALSFRS-R, and ALSFRS-R subscores in longitudinal and survival models, to determine whether subscore analysis enhances the precision of the instrument. METHODS: All cases with ALSFRS-R scores on the Irish ALS register were included...
November 25, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
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