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https://www.readbyqxmd.com/read/28100104/theoretical-predication-of-temperature-effects-on-accommodative-processes-in-simulated-amyotrophic-lateral-sclerosis-during-hypothermia-and-hyperthermia
#1
D I Stephanova, A Kossev
Electrotonic potentials allow the accommodative processes to long-lasting subthreshold polarizing stimuli to be assessed. The present study investigates such potentials in previously simulated cases of amyotrophic lateral sclerosis, termed as ALS1, ALS2 and ALS3, respectively, when the temperature is changed during hypothermia ([Formula: see text]C) and hyperthermia ([Formula: see text]C). The ALS cases are modeled as three progressively severe uniform axonal dysfunctions along the human motor nerve fiber which is simulated by our temperature-dependent multi-layered numerical model...
January 18, 2017: Journal of Integrative Neuroscience
https://www.readbyqxmd.com/read/28093823/sleep-and-its-associations-with-perceived-and-objective-cognitive-impairment-in-individuals-with-multiple-sclerosis
#2
Abbey J Hughes, Brett A Parmenter, Jodie K Haselkorn, Jesus F Lovera, Dennis Bourdette, Eilis Boudreau, Michelle H Cameron, Aaron P Turner
Problems with sleep and cognitive impairment are common among people with multiple sclerosis (MS). The present study examined the relationship between self-reported sleep and both objective and perceived cognitive impairment in MS. Data were obtained from the baseline assessment of a multi-centre intervention trial (NCT00841321). Participants were 121 individuals with MS. Nearly half (49%) of participants met the criteria for objective cognitive impairment; however, cognitively impaired and unimpaired participants did not differ on any self-reported sleep measures...
January 17, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/28086792/irish-general-practitioner-attitudes-toward-decriminalisation-and-medical-use-of-cannabis-results-from-a-national-survey
#3
Des Crowley, Claire Collins, Ide Delargy, Eamon Laird, Marie Claire Van Hout
BACKGROUND: Governmental debate in Ireland on the de facto decriminalisation of cannabis and legalisation for medical use is ongoing. A cannabis-based medicinal product (Sativex®) has recently been granted market authorisation in Ireland. This unique study aimed to investigate Irish general practitioner (GP) attitudes toward decriminalisation of cannabis and assess levels of support for use of cannabis for therapeutic purposes (CTP). METHODS: General practitioners in the Irish College of General Practitioner (ICGP) database were invited to complete an online survey...
January 13, 2017: Harm Reduction Journal
https://www.readbyqxmd.com/read/28069095/deficits-in-social-cognition-an-unveiled-signature-of-multiple-sclerosis
#4
Moussa A Chalah, Samar S Ayache
BACKGROUND AND OBJECTIVES: Multiple sclerosis (MS) is a chronic progressive inflammatory disease of the central nervous system, representing the primary cause of non-traumatic disability in young adults. Cognitive dysfunction can affect patients at any time during the disease process and might alter the six core functional domains. Social cognition is a multi-component construct that includes the theory of mind, empathy and social perception of emotions from facial, bodily and vocal cues...
January 10, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28058539/validation-of-the-ucla-scleroderma-clinical-trial-consortium-gastrointestinal-tract-instrument-2-0-in-english-and-chinese-speaking-patients-in-a-multi-ethnic-singapore-systemic-sclerosis-cohort
#5
Andrea Hsiu Ling Low, Xiaohui Xin, Weng Giap Law, Gim Gee Teng, Amelia Santosa, Anita Lim, Grace Chan, Swee Cheng Ng, Julian Thumboo
The aim of this study was to (1) translate the Gastrointestinal Tract Instrument (GIT) 2.0 from English to Chinese and (2) validate both versions in a multi-ethnic systemic sclerosis cohort in Singapore (SCORE). The English GIT2.0 was translated to Chinese using a standard forward-backward translation approach. Psychometric evaluation of the GIT2.0 included internal consistency reliability (using Cronbach's alpha), test-retest reliability (using intra-class correlation coefficient (ICC)), scale level factor analysis, and construct validity (using Spearman correlation) against the modified Scleroderma Health Assessment Questionnaire (S-HAQ) and the SF-36 v2...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28058507/als-ftld-experimental-models-and-reality
#6
REVIEW
Rachel H Tan, Yazi D Ke, Lars M Ittner, Glenda M Halliday
Amyotrophic lateral sclerosis is characterised by a loss of upper and lower motor neurons and characteristic muscle weakness and wasting, the most common form being sporadic disease with neuronal inclusions containing the tar DNA-binding protein 43 (TDP-43). Frontotemporal lobar degeneration is characterised by atrophy of the frontal and/or temporal lobes, the most common clinical form being the behavioural variant, in which neuronal inclusions containing either TDP-43 or 3-repeat tau are most prevalent. Although the genetic mutations associated with these diseases have allowed various experimental models to be developed, the initial genetic forms identified remain the most common models employed to date...
January 5, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28056476/-advanced-systemic-sclerosis-endovascular-reconstruction-of-the-aortic-bifurcation-for-limb-salvage
#7
Florian Stein, Lars Kamper, Konstantinos Meletiadis, Ulrich Kusenack, Patrick Haage
History and admission findings A 69-year-old patient was initially hospitalized because of a 1.5 cm ulceration at the back of the right foot which had existed for the last year and become increasingly swollen and painful. Medical history revealed PmScl-positive systemic sclerosis presenting with a massive calcinosis cutis, advanced pulmonary fibrosis and peripheral artery disease. Examinations Inflammatory markers were normal. MR-Angiography of the lower extremities revealed a multi-segmental high-grade stenosis of the aortic bifurcation due to extensively calcified plaques...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28046971/th-cd-206-01-expectation-maximization-algorithm-based-tissue-mixture-quantification-for-perfusion-mri
#8
H Han, Z Liang, L Li, L Xing
PURPOSE: To investigate the feasibility of estimating the tissue mixture perfusions and quantifying cerebral blood flow change in arterial spin labeled (ASL) perfusion MR images. METHODS: The proposed perfusion MR image analysis framework consists of 5 steps: (1) Inhomogeneity correction was performed on the T1- and T2-weighted images, which are available for each studied perfusion MR dataset. (2) We used the publicly available FSL toolbox to strip off the non-brain structures from the T1- and T2-weighted MR images...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28013293/deficient-tsc1-tsc2-complex-suppression-of-sox9-osteopontin-akt-signalling-cascade-constrains-tumour-growth-in-tuberous-sclerosis-complex
#9
Fuquan Jin, Keguo Jiang, Shuang Ji, Li Wang, Zhaofei Ni, Fuqiang Huang, Chunjia Li, Rongrong Chen, Hongbing Zhang, Zhongdong Hu, Xiaojun Zha
Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder featured with multi-organ benign tumours. Disruption of TSC1/TSC2 complex suppression on mammalian/mechanistic target of rapamycin (mTOR) signalling causes TSC. Hyperactive mTOR-mediated negative feedback regulation of AKT partially contributes to the benign nature of TSC-associated tumours. In this study, we demonstrated that osteopontin (OPN) was dramatically reduced by loss of TSC1/TSC2 complex in Tsc2-null mouse embryonic fibroblasts (MEFs), rat uterine leiomyoma-derived Tsc2-deficient cells, genetically modified mouse TSC models, and clinical samples...
December 23, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28009132/tract-specific-atrophy-in-focal-epilepsy-disease-genetics-or-seizures
#10
David N Vaughan, David Raffelt, Evan Curwood, Meng-Han Tsai, Jacques-Donald Tournier, Alan Connelly, Graeme D Jackson
OBJECTIVE: To investigate whether genetics, underlying pathology or repeated seizures contribute to atrophy in specific white matter tracts. METHODS: Medically-refractory unilateral temporal lobe epilepsy with hippocampal sclerosis (HS-TLE, n=26) was studied as an archetype of focal epilepsy, using fixel-based analysis of diffusion-weighted imaging. A genetic effect was assessed in first-degree relatives of HS-TLE who did not have epilepsy themselves (HS-1°Rel; n=26)...
December 23, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/28009079/acute-disseminated-encephalomyelitis-in-china-singapore-and-japan-a-comparison-with-the-usa
#11
D L H Koelman, D C Benkeser, Y Xu, S X Neo, K Tan, M Katsuno, G Sobue, J Natsume, S Chahin, S S Mar, A Venkatesan, T Chitnis, G M Hoganson, A K Yeshokumar, P Barreras, B Majmudar, M Carone, F J Mateen
BACKGROUND AND PURPOSE: Ethnicity-related differences in the incidence of acute disseminated encephalomyelitis (ADEM) and other demyelinating diseases including multiple sclerosis and neuromyelitis optica spectrum disorders have been reported. Little is reported on the influence of ethnicity and geographical location in ADEM. METHODS: Medical records of patients who presented with ADEM (ICD-9 323.61 and 323.81) at large referral hospitals in China, Singapore and Japan (years 1992-2015) were retrospectively reviewed and data were collected in a centralized database...
February 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28004651/autophagy-a-brief-overview-in-perspective-of-dermatology
#12
REVIEW
Rahul Nagar
Autophagy, literally meaning "self-eating," is an intracellular catabolic process of delivering cytosol and/or its specific content to the lysosomes for degradation.The resulting macromolecular constituents are recycled and utilized again by the cells. Basal level autophagy plays an important role in cellular homeostasis through the elimination of the old or damaged organelles, as well as aggregated intracellular proteins. Autophagy refers to sequestration of intact organelles along with a portion of cytosol, into a double-or multi-membrane structure known as phagophore, which elongates, and after closure, forms a vesicular structure known as the autophagosome...
December 20, 2016: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/27995062/magnetic-susceptibility-in-the-deep-layers-of-the-primary-motor-cortex-in-amyotrophic-lateral-sclerosis
#13
M Costagli, G Donatelli, L Biagi, E Caldarazzo Ienco, G Siciliano, M Tosetti, M Cosottini
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurological disorder that entails degeneration of both upper and lower motor neurons. The primary motor cortex (M1) in patients with upper motor neuron (UMN) impairment is pronouncedly hypointense in Magnetic Resonance (MR) T2* contrast. In the present study, 3D gradient-recalled multi-echo sequences were used on a 7 Tesla MR system to acquire T2*-weighted images targeting M1 at high spatial resolution. MR raw data were used for Quantitative Susceptibility Mapping (QSM)...
2016: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27930937/advances-in-myelinating-glial-cell-development
#14
REVIEW
Amy L Herbert, Kelly R Monk
In the vertebrate nervous system, the fast conduction of action potentials is potentiated by the myelin sheath, a multi-lamellar, lipid-rich structure that also provides vital trophic and metabolic support to axons. Myelin is elaborated by the plasma membrane of specialized glial cells, oligodendrocytes in the central nervous system (CNS) and Schwann cells (SCs) in the peripheral nervous system (PNS). The diseases that result from damage to myelin or glia, including multiple sclerosis and Charcot-Marie-Tooth disease, underscore the importance of these cells for human health...
December 5, 2016: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/27918067/systematic-approach-to-understanding-the-pathogenesis-of-systemic-sclerosis
#15
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/27911122/an-update-on-the-use-of-cerebrospinal-fluid-analysis-as-a-diagnostic-tool-in-multiple-sclerosis
#16
Matteo Gastaldi, Elisabetta Zardini, Diego Franciotta
Intrathecal B-lymphocyte activation is a hallmark of multiple sclerosis (MS), a multi-factorial inflammatory-demyelinating disease of the central nervous system. Such activation has a counterpart in the cerebrospinal fluid (CSF) oligoclonal IgG bands (OCB), whose diagnostic role in MS has been downgraded within the current McDonald's criteria. With a theoretico-practical approach, the authors review the physiopathological basis of the CSF dynamics, and the state-of-the-art of routine CSF analysis and CSF biomarkers in MS...
January 2017: Expert Review of Molecular Diagnostics
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/27885448/cognitive-dysfunction-in-patients-with-multiple-sclerosis-treated-with-first-line-disease-modifying-therapy-a-multi-center-controlled-study-using-the-bicams-battery
#18
Bilge Piri Cinar, Görkem Kösehasanoğulları, Pinar Yigit, Serkan Ozakbas
Multiple sclerosis (MS) can impair cognitive functions even in the early stages. The Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) battery is very short and highly sensitive and can be used to evaluate cognitive status in the disease. Several clinical trials have shown beneficial effects of disease-modifying drugs (DMDs) on long-term cognitive measures which may even reduce cognitive deficits in MS patients. Relapsing remitting MS patients using DMDs were enrolled in the study and monitored for 12 months...
November 24, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27872810/the-association-between-intra-and-juxta-cortical-pathology-and-cognitive-impairment-in-multiple-sclerosis-by-quantitative-t2-mapping-at-7%C3%A2-t-mri
#19
Céline Louapre, Sindhuja T Govindarajan, Costanza Giannì, Nancy Madigan, A Scott Nielsen, Jacob A Sloane, Revere P Kinkel, Caterina Mainero
Using quantitative T2* at 7 Tesla (T) magnetic resonance imaging, we investigated whether impairment in selective cognitive functions in multiple sclerosis (MS) can be explained by pathology in specific areas and/or layers of the cortex. Thirty-one MS patients underwent neuropsychological evaluation, acquisition of 7 T multi-echo T2* gradient-echo sequences, and 3 T anatomical images for cortical surfaces reconstruction. Seventeen age-matched healthy subjects served as controls. Cortical T2* maps were sampled at various depths throughout the cortex and juxtacortex...
2016: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27872808/delineation-of-cortical-pathology-in-multiple-sclerosis-using-multi-surface-magnetization-transfer-ratio-imaging
#20
David A Rudko, Mishkin Derakhshan, Josefina Maranzano, Kunio Nakamura, Douglas L Arnold, Sridar Narayanan
The purpose of our study was to evaluate the utility of measurements of cortical surface magnetization transfer ratio (csMTR) on the inner, mid and outer cortical boundaries as clinically accessible biomarkers of cortical gray matter pathology in multiple sclerosis (MS). Twenty-five MS patients and 12 matched controls were recruited from the MS Clinic of the Montreal Neurological Institute. Anatomical and magnetization transfer ratio (MTR) images were acquired using 3 Tesla MRI at baseline and two-year time-points...
2016: NeuroImage: Clinical
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