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https://www.readbyqxmd.com/read/28746029/early-tissue-damage-and-microstructural-reorganization-predict-disease-severity-in-experimental-epilepsy
#1
Philipp Janz, Niels Schwaderlapp, Katharina Heining, Ute Häussler, Jan G Korvink, Dominik von Elverfeldt, Jürgen Hennig, Ulrich Egert, Pierre LeVan, Carola A Haas
Mesial temporal lobe epilepsy (mTLE) is the most common focal epilepsy in adults and is often refractory to medication. So far, resection of the epileptogenic focus represents the only curative therapy. It is unknown whether pathological processes preceding epilepsy onset are indicators of later disease severity. Using longitudinal multi-modal MRI, we monitored hippocampal injury and tissue reorganization during epileptogenesis in a mouse mTLE model. The prognostic value of MRI biomarkers was assessed by retrospective correlations with pathological hallmarks Here, we show for the first time that the extent of early hippocampal neurodegeneration and progressive microstructural changes in the dentate gyrus translate to the severity of hippocampal sclerosis and seizure burden in chronic epilepsy...
July 26, 2017: ELife
https://www.readbyqxmd.com/read/28744108/patient-adherence-to-subcutaneous-ifn-beta-1a-injections-using-the-rebismart-%C3%A2-injection-device-a-retrospective-real-world-study-among-dutch-and-german-patients-with-multiple-sclerosis
#2
Marieke Krol, Gert de Voer, Ulrike Osowski
PURPOSE: Long-term treatment adherence among patients with multiple sclerosis (MS) is a general concern, with an established correlation with clinical efficacy. Closely monitoring patients' treatment behavior may have a beneficial effect on adherence. This study assessed adherence, in daily life, to subcutaneous (sc) IFN beta-1a, self-administered using the RebiSmart(®) electronic injection device (the IFN beta-Ia autoinjector device), in patients with MS. PATIENTS AND METHODS: This was a retrospective observational study analyzing treatment adherence based on injection data, eg, injection date and dose, extracted from the IFN beta-Ia autoinjector devices collected from patients in Germany and the Netherlands...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28742871/comprehensive-immune-profiling-reveals-substantial-immune-system-alterations-in-a-subset-of-patients-with-amyotrophic-lateral-sclerosis
#3
Michael P Gustafson, Nathan P Staff, Svetlana Bornschlegl, Greg W Butler, Mary L Maas, Mohamed Kazamel, Adeel Zubair, Dennis A Gastineau, Anthony J Windebank, Allan B Dietz
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with a median lifespan of 2-3 years after diagnosis. There are few meaningful treatments that alter progression in this disease. Preclinical and clinical studies have demonstrated that neuroinflammation may play a key role in the progression rate of ALS. Despite this, there are no validated biomarkers of neuroinflammation for use in clinical practice or clinical trials. Biomarkers of neuroinflammation could improve patient management, provide new therapeutic targets, and possibly help stratify clinical trial selection and monitoring...
2017: PloS One
https://www.readbyqxmd.com/read/28732757/multimodal-exercise-training-in-multiple-sclerosis-a-randomized-controlled-trial-in-persons-with-substantial-mobility-disability
#4
Brian M Sandroff, Rachel E Bollaert, Lara A Pilutti, Melissa L Peterson, Tracy Baynard, Bo Fernhall, Edward McAuley, Robert W Motl
BACKGROUND: Mobility disability is a common, debilitating feature of multiple sclerosis (MS). Exercise training has been identified as an approach to improve MS-related mobility disability. However, exercise randomized controlled trials (RCTs) on mobility in MS have generally not selectively targeted those with the onset of irreversible mobility disability. OBJECTIVES: The current multi-site RCT compared the efficacy of 6-months of supervised, multimodal exercise training with an active control condition for improving mobility, gait, physical fitness, and cognitive outcomes in persons with substantial MS-related mobility disability...
July 19, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28700179/-clinical-manifestations-and-therapeutic-perspectives-in-tuberous-sclerosis-a-rare-multi-organ-genetic-disease-with-renal-involvement
#5
Giorgia Apollonia Caloro, Marta Spinelli, Nicla Campobasso, Tommaso Esposito, Loreto Gesualdo, Vincenzo Montinaro
Tuberous sclerosis is a rare genetic disease with multiple organ involvement. Renal involvement is manifested by the presence of angiomyolipomas and cysts. Angiomyolipomas due to the progressive increase in size over time can complicate by bleeding that can lead to severe retroperitoneal hemorrhages. Hence, it is important to perform an active surveillance of the masses and to institute therapeutic attempts in order to manage or prevent any complication. We present three clinical cases of three women with various clinical presentations and treated in different ways...
June 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28699215/automated-t2-relaxometry-of-the-hippocampus-for-temporal-lobe-epilepsy
#6
Gavin P Winston, Sjoerd B Vos, Jane L Burdett, M Jorge Cardoso, Sebastien Ourselin, John S Duncan
OBJECTIVE: Hippocampal sclerosis (HS), the most common cause of refractory temporal lobe epilepsy, is associated with hippocampal volume loss and increased T2 signal. These can be identified on quantitative imaging with hippocampal volumetry and T2 relaxometry. Although hippocampal segmentation for volumetry has been automated, T2 relaxometry currently involves subjective and time-consuming manual delineation of regions of interest. In this work, we develop and validate an automated technique for hippocampal T2 relaxometry...
July 12, 2017: Epilepsia
https://www.readbyqxmd.com/read/28689102/monthly-methylprednisolone-in-combination-with-interferon-beta-or-glatiramer-acetate-for-relapsing-remitting-multiple-sclerosis-a-multicentre-single-blind-prospective-trial
#7
Serkan Ozakbas, Bilge Piri Cinar, Gorkem Kosehasanoğullari, Turhan Kahraman, Didem Oz, Behice Bircan Kursun
OBJECTIVES: Multiple sclerosis is usually clinically characterized by repeated subacute relapses followed by remissions. Corticosteroids are used for relapses, and this treatment has been shown to increase the speed of recovery from these. We aimed to evaluate the efficacy and safety of pulsed methylprednisolone given every month as an add-on therapy to interferon beta or glatiramer acetate in patients with relapsing-remitting multiple sclerosis. PATIENTS AND METHODS: This was a multi-center, examiner-blinded, prospective study...
June 27, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28676143/sensitivity-of-multi-shell-noddi-to-multiple-sclerosis-white-matter-changes-a-pilot-study
#8
Torben Schneider, W Brownlee, H Zhang, Olga Ciccarelli, David H Miller, Claudia Gandini Wheeler-Kingshott
Diffusion tensor imaging (DTI) is sensitive to white matter (WM) damage in multiple sclerosis (MS), not only in focal lesions but also in the normal-appearing WM (NAWM). However, DTI indices can also be affected by natural spatial variation in WM, as seen in crossing and dispersing white matter fibers. Neurite orientation dispersion and density imaging (NODDI) is an advanced diffusion-weighted imaging technique that provides distinct indices of fiber density and dispersion. We performed NODDI of lesion tissue and NAWM in five MS patients and five controls, comparing the technique with traditional DTI...
April 2017: Functional Neurology
https://www.readbyqxmd.com/read/28669906/thalamus-segmentation-using-multi-modal-feature-classification-validation-and-pilot-study-of-an-age-matched-cohort
#9
Jeffrey Glaister, Aaron Carass, Tziona NessAiver, Joshua V Stough, Shiv Saidha, Peter A Calabresi, Jerry L Prince
Automatic segmentation of the thalamus can be used to measure differences and track changes in thalamic volume that may occur due to disease, injury or normal aging. An automatic thalamus segmentation algorithm incorporating features from diffusion tensor imaging (DTI) and thalamus priors constructed from multiple atlases is proposed. Multiple atlases with corresponding manual thalamus segmentations are registered to the target image and averaged to generate the thalamus prior. At each voxel in a region of interest around the thalamus, a multidimensional feature vector that includes the thalamus prior as well as a set of DTI features, including fractional anisotropy, mean diffusivity, and fiber orientation is formed...
June 29, 2017: NeuroImage
https://www.readbyqxmd.com/read/28663780/new-insights-into-the-pathogenesis-and-prevention-of-tuberous-sclerosis-associated-neuropsychiatric-disorders-tand
#10
REVIEW
Tanjala T Gipson, Michael V Johnston
Tuberous sclerosis complex (TSC) is a multi-system disorder resulting from mutations in either the TSC1 or TSC2 genes leading to hyperactivation of mechanistic target of rapamycin (mTOR) signaling. TSC is commonly associated with autism (61%), intellectual disability (45%), and behavioral, psychiatric, intellectual, academic, neuropsychological, and psychosocial difficulties that are collectively referred to as TSC-associated neuropsychiatric disorders (TAND). More than 90% of children with TSC have epilepsy, including infantile spasms, and early onset of seizures, especially infantile spasms, is associated with greater impairment in intellectual development compared with individuals with TSC without seizures...
2017: F1000Research
https://www.readbyqxmd.com/read/28655471/early-inflammatory-players-in-cutanous-fibrosis
#11
REVIEW
Verena Raker, Jessica Haub, Ana Stojanovic, Adelheid Cerwenka, Detlef Schuppan, Kerstin Steinbrink
Systemic sclerosis (SSc) is one of the most complex systemic autoimmune diseases with multi-organ involvement and heterogeneous clinical manifestations. The exact etiology of SSc is still unknown. However, identified target structures are components of endothelial cells, the innate/adaptive immune systems and fibroblasts, resulting in the hallmarks of the disease in form of inflammation/autoimmunity, vasculopathy and fibrosis of the skin and internal organs. There has been a large body of evidence that the adaptive immune system with autoreactive T and B cells producing autoantibodies plays a central role in the pathogenesis of SSc but the role of earlier pathogenic processes involving the innate immune system, is far from being understood...
June 15, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28646232/inhibition-of-erk1-2-restores-gsk3%C3%AE-activity-and-protein-synthesis-levels-in-a-model-of-tuberous-sclerosis
#12
Rituraj Pal, Vitaliy V Bondar, Carolyn J Adamski, George G Rodney, Marco Sardiello
Tuberous sclerosis (TS) is a multi-organ autosomal dominant disorder that is best characterized by neurodevelopmental deficits and the presence of benign tumors. TS pathology is caused by mutations in tuberous sclerosis complex (TSC) genes and is associated with insulin resistance, decreased glycogen synthase kinase 3β (GSK3β) activity, activation of the mammalian target of rapamycin complex 1 (mTORC1), and subsequent increase in protein synthesis. Here, we show that extracellular signal-regulated kinases (ERK1/2) respond to insulin stimulation and integrate insulin signaling to phosphorylate and thus inactivate GSK3β, resulting in increased protein synthesis that is independent of Akt/mTORC1 activity...
June 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28637128/maximizing-gait-and-balance-behaviors-and-decision-making-processes-of-persons-with-multiple-sclerosis-and-physical-therapists
#13
Elissa Held Bradford, Marcia Finlayson, Andrea White Gorman, Joanne Wagner
PURPOSE: To describe the behavioral decisions used by persons with multiple sclerosis (MS) and physical therapists to maximize gait and balance following outpatient physical therapy. METHODS: A multi-method case series with seven matched pairs (persons with MS-physical therapists). Quota sampling maximized variability among persons with MS (disease steps score range 3-6). Three of the four physical therapists were MS or neurology certified. Persons with MS completed a phone survey, follow-up interview, and standardized questionnaires...
February 7, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28636179/discontinuation-and-non-publication-of-neurodegenerative-disease-trials-a-cross-sectional-analysis
#14
J D Stefaniak, T C H Lam, N E Sim, R Al-Shahi Salman, D P Breen
BACKGROUND AND PURPOSE: Trial discontinuation and non-publication represent major sources of research waste in clinical medicine. No previous studies have investigated non-dissemination bias in clinical trials of neurodegenerative diseases. METHODS: ClinicalTrials.gov was searched for all randomized, interventional, phase II-IV trials that were registered between 1 January 2000 and 31 December 2009 and included adults with Alzheimer's disease, motor neurone disease, multiple sclerosis or Parkinson's disease...
June 21, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28619436/tumefactive-demyelinating-lesions-a-comprehensive-review
#15
REVIEW
Hussein Algahtani, Bader Shirah, Ali Alassiri
Tumefactive multiple sclerosis or tumefactive demyelinating lesion (TDL) is one of the rare variants of multiple sclerosis (MS) posing a diagnostic challenge and a therapeutic enigma since it is difficult to distinguish from a true central nervous system (CNS) neoplasm or other CNS lesions on magnetic resonance imaging (MRI). The prevalence of TDL is estimated to be 1-3/1000 cases of MS with an annual incidence of 0.3/100,000. This could be an underestimate due to unavailability of a global MS registry and under-reporting of this condition...
May 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28591490/region-specific-atrophy-of-precentral-gyrus-in-patients-with-amyotrophic-lateral-sclerosis
#16
Yuanyuan Qin, Shun Zhang, Rifeng Jiang, Fei Gao, Xiaoying Tang, Wenzhen Zhu
PURPOSE: To assess the region-specific atrophy of precentral gyrus (PrCG) and its correlation to clinical function score in amyotrophic lateral sclerosis (ALS). MATERIALS AND METHODS: Twenty-eight patients with sporadic ALS and 28 healthy controls underwent high-resolution 3D T1-BRAVO magnetic resonance imaging at 3T. The bilateral PrCG segmentations were automatically obtained from a validated segmentation pipeline based on diffeomorphic multi-atlas likelihood fusion...
June 7, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28586096/spinal-cord-multi-parametric-magnetic-resonance-imaging-for-survival-prediction-in-amyotrophic-lateral-sclerosis
#17
G Querin, M M El Mendili, T Lenglet, S Delphine, V Marchand-Pauvert, H Benali, P-F Pradat
BACKGROUND AND PURPOSE: Assessing survival is a critical issue in patients with amyotrophic lateral sclerosis (ALS). Neuroimaging seems to be promising in the assessment of disease severity and several studies also suggest a strong relationship between spinal cord (SC) atrophy described by magnetic resonance imaging (MRI) and disease progression. The aim of the study was to determine the predictive added value of multimodal SC MRI on survival. METHODS: Forty-nine ALS patients were recruited and clinical data were collected...
August 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28580337/neural-network-based-learning-kernel-for-automatic-segmentation-of-multiple-sclerosis-lesions-on-magnetic-resonance-images
#18
H Khastavaneh, H Ebrahimpour-Komleh
BACKGROUND: Multiple Sclerosis (MS) is a degenerative disease of central nervous system. MS patients have some dead tissues in their brains called MS lesions. MRI is an imaging technique sensitive to soft tissues such as brain that shows MS lesions as hyper-intense or hypo-intense signals. Since manual segmentation of these lesions is a laborious and time consuming task, automatic segmentation is a need. MATERIALS AND METHODS: In order to segment MS lesions, a method based on learning kernels has been proposed...
June 2017: Journal of Biomedical Physics & Engineering
https://www.readbyqxmd.com/read/28569182/a-pharmacogenetic-signature-of-high-response-to-copaxone-in-late-phase-clinical-trial-cohorts-of-multiple-sclerosis
#19
Colin J Ross, Fadi Towfic, Jyoti Shankar, Daphna Laifenfeld, Mathis Thoma, Matthew Davis, Brian Weiner, Rebecca Kusko, Ben Zeskind, Volker Knappertz, Iris Grossman, Michael R Hayden
BACKGROUND: Copaxone is an efficacious and safe therapy that has demonstrated clinical benefit for over two decades in patients with relapsing forms of multiple sclerosis (MS). On an individual level, patients show variability in their response to Copaxone, with some achieving significantly higher response levels. The involvement of genes (e.g., HLA-DRB1*1501) with high inter-individual variability in Copaxone's mechanism of action (MoA) suggests the potential contribution of genetics to treatment response...
May 31, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28560158/application-and-evaluation-of-noddi-in-the-cervical-spinal-cord-of-multiple-sclerosis-patients
#20
Samantha By, Junzhong Xu, Bailey A Box, Francesca R Bagnato, Seth A Smith
INTRODUCTION: There is a need to develop imaging methods sensitive to axonal injury in multiple sclerosis (MS), given the prominent impact of axonal pathology on disability and outcome. Advanced multi-compartmental diffusion models offer novel indices sensitive to white matter microstructure. One such model, neurite orientation dispersion and density imaging (NODDI), is sensitive to neurite morphology, providing indices of apparent volume fractions of axons (vin), isotropic water (viso) and the dispersion of fibers about a central axis (orientation dispersion index, ODI)...
2017: NeuroImage: Clinical
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