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Inclusion body disease

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https://www.readbyqxmd.com/read/29166468/lafora-and-tr%C3%A3-tiakoff-the-naming-of-the-inclusion-bodies-discovered-by-lewy
#1
Eliasz Engelhardt
Fritz Heinrich Jakob Lewy described, for the first time, in 1912, novel peculiar inclusions in neurons of certain brain nuclei in patients with Paralysis agitans, and compared his finding to the amyloid bodies described by Lafora one year before. Gonzalo Rodriguez Lafora studied one patient with Paralysis agitans, in 1913, and recognized, described, and depicted structures identical to those previously reported by Lewy. He was the first to acknowledge Lewy's finding, and also the first to name such inclusions after the discoverer - cuerpos intracelulares de Lewy (Lewy bodies)...
October 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/29164600/18f-pet-with-florbetaben-for-the-early-diagnosis-of-alzheimer-s-disease-dementia-and-other-dementias-in-people-with-mild-cognitive-impairment-mci
#2
REVIEW
Gabriel Martínez, Robin Wm Vernooij, Paulina Fuentes Padilla, Javier Zamora, Leon Flicker, Xavier Bonfill Cosp
BACKGROUND: (18)F-florbetaben uptake by brain tissue, measured by positron emission tomography (PET), is accepted by regulatory agencies like the Food and Drug Administration (FDA) and the European Medicine Agencies (EMA) for assessing amyloid load in people with dementia. Its added value is mainly demonstrated by excluding Alzheimer's pathology in an established dementia diagnosis. However, the National Institute on Aging and Alzheimer's Association (NIA-AA) revised the diagnostic criteria for Alzheimer's disease and confidence in the diagnosis of mild cognitive impairment (MCI) due to Alzheimer's disease may be increased when using some amyloid biomarkers tests like (18)F-florbetaben...
November 22, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29164033/change-in-functional-impairment-disability-and-quality-of-life-following-operative-treatment-for-degenerative-cervical-myelopathy-a-systematic-review-and-meta-analysis
#3
REVIEW
Michael G Fehlings, Lindsay A Tetreault, Shekar Kurpad, Darrel S Brodke, Jefferson R Wilson, Justin S Smith, Paul M Arnold, Erika D Brodt, Joseph R Dettori
Study Design: Systematic review. Objectives: The primary objective of this systematic review was to define the change in impairment, disability, and pain following surgical intervention in patients with degenerative cervical myelopathy (DCM). Secondary objectives included to assess the impact of preoperative disease severity and duration of symptoms on outcomes and to summarize complications associated with surgery. Methods: A systematic literature search was conducted to identify prospective studies evaluating the effectiveness and safety of operative treatment in patients with DCM...
September 2017: Global Spine Journal
https://www.readbyqxmd.com/read/29159551/long-term-over-10%C3%A2-years-retrospective-follow-up-of-laparoscopic-adjustable-gastric-banding
#4
Dvir Froylich, Tamar Abramovich-Segal, Guy Pascal, Ivy Haskins, Boaz Appel, Naama Kafry, David Hazzan
BACKGROUND: Laparoscopic adjustable gastric banding (LAGB) placements have progressively decreased in recent years. This is related to poor long-term weight loss outcomes and necessity for revision or removal of these bands. Long-term outcome results following LAGB are limited. The aim of our study was to determine the long-term outcome after LAGB at our institution. OBJECTIVES: The aim of our study was to determine the long-term outcome after LAGB at our institution...
October 6, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/29158232/development-and-validation-of-qdiabetes-2018-risk-prediction-algorithm-to-estimate-future-risk-of-type-2-diabetes-cohort-study
#5
Julia Hippisley-Cox, Carol Coupland
Objectives To derive and validate updated QDiabetes-2018 prediction algorithms to estimate the 10 year risk of type 2 diabetes in men and women, taking account of potential new risk factors, and to compare their performance with current approaches.Design Prospective open cohort study.Setting Routinely collected data from 1457 general practices in England contributing to the QResearch database: 1094 were used to develop the scores and a separate set of 363 were used to validate the scores.Participants 11...
November 20, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/29157151/health-benefits-of-hard-martial-arts-in-adults-a-systematic-review
#6
Sandra Origua Rios, Jennifer Marks, Isaac Estevan, Lisa M Barnett
Participation in organized sports is promoted as a means of increasing physical activity levels and reducing chronic disease risk in adults. Hard martial arts practice (i.e. using body contact techniques), has gained in popularity over time. This review explores the evidence for health benefits of "hard" martial arts practice within the adult population. A systematic electronic database search was conducted, and quality assessments applied the Effective Public Health Practice Project tool. Twenty-eight studies met the inclusion criteria, examining balance, cognitive function, muscular skeletal status, psychological, cardiovascular fitness, and metabolic effects...
November 21, 2017: Journal of Sports Sciences
https://www.readbyqxmd.com/read/29155947/dementia-after-moderate-severe-traumatic-brain-injury-coexistence-of-multiple-proteinopathies
#7
Kimbra Kenney, Diego Iacono, Brian L Edlow, Douglas I Katz, Ramon Diaz-Arrastia, Kristen Dams-O'Connor, Daniel H Daneshvar, Allison Stevens, Allison L Moreau, Lee S Tirrell, Ani Varjabedian, Anastasia Yendiki, Andre van der Kouwe, Azma Mareyam, Jennifer A McNab, Wayne A Gordon, Bruce Fischl, Ann C McKee, Daniel P Perl
We report the clinical, neuroimaging, and neuropathologic characteristics of 2 patients who developed early onset dementia after a moderate-severe traumatic brain injury (TBI). Neuropathological evaluation revealed abundant β-amyloid neuritic and cored plaques, diffuse β-amyloid plaques, and frequent hyperphosphorylated-tau neurofibrillary tangles (NFT) involving much of the cortex, including insula and mammillary bodies in both cases. Case 1 additionally showed NFTs in both the superficial and deep cortical layers, occasional perivascular and depth-of-sulci NFTs, and parietal white matter rarefaction, which corresponded with decreased parietal fiber tracts observed on ex vivo MRI...
November 16, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29152438/barriers-to-timely-completion-of-radiation-therapy-in-patients-with-cervical-cancer-in-an-urban-tertiary-care-center
#8
Justin Cohen, Amy Harper, Elizabeth M Nichols, Gautam G Rao, Pranshu Mohindra, Dana Marie Roque
Background In 2017, there will be an estimated 12,820 women diagnosed with cervical cancer in the United States, causing an estimation of 4,210 deaths. Among U.S. women, there is a 33% greater incidence and 71% higher cervical cancer mortality in high-poverty counties when compared to low-income counties [1]. In those dispositioned to chemoradiation, treatment time of less than eight weeks is associated with compromised pelvic control. We sought to identify patient or disease characteristics and socioeconomic or psychosocial barriers that contribute to delays in treatment completion in order to formulate new policies to address these needs...
September 12, 2017: Curēus
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#9
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29150913/molecular-epidemiology-of-goat-pox-viruses
#10
P Roy, S Jaisree, S Balakrishnan, K Senthilkumar, R Mahaprabhu, A Mishra, B Maity, T K Ghosh, A P Karmakar
Goat pox disease outbreaks were observed in different places affecting Black Bengal Goats in West Bengal (WB) and Tellicherry, Vembur and non-descriptive breeds in Tamil Nadu (TN) causing severe lesions and mortality up to 30%. Clinical specimens from all the outbreaks were screened by polymerase chain reaction followed by restriction fragment length polymorphism (PCR-RFLP) and confirmed the diseases as Goat Pox. Virus isolation in Vero cell line was done with randomly selected ten samples, cytopathic effects (CPE) characterized by syncytia and intracytoplasmic inclusion bodies were observed after several blind passages...
November 18, 2017: Transboundary and Emerging Diseases
https://www.readbyqxmd.com/read/29146481/central-obesity-is-the-key-component-in-the-association-of-metabolic-syndrome-with-left-ventricular-global-longitudinal-strain-impairment
#11
Wilson Cañon-Montañez, Angela B S Santos, Lisandra A Nunes, Julio C G Pires, Claudia M V Freire, Antonio L P Ribeiro, José G Mill, Marina Bessel, Bruce B Duncan, Maria Inês Schmidt, Murilo Foppa
INTRODUCTION AND OBJECTIVES: Subclinical systolic dysfunction is one of the proposed mechanisms for increased cardiovascular risk associated with metabolic syndrome (MS). This study investigated the association between MS and impaired left ventricular global longitudinal strain (GLS) and the role of each MS criteria in this association. METHODS: We analyzed a random sample of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil) after excluding participants with prevalent heart disease...
November 13, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/29146301/association-of-sputum-and-blood-eosinophil-concentrations-with-clinical-measures-of-copd-severity-an-analysis-of-the-spiromics-cohort
#12
Annette T Hastie, Fernando J Martinez, Jeffrey L Curtis, Claire M Doerschuk, Nadia N Hansel, Stephanie Christenson, Nirupama Putcha, Victor E Ortega, Xingnan Li, R Graham Barr, Elizabeth E Carretta, David J Couper, Christopher B Cooper, Eric A Hoffman, Richard E Kanner, Eric Kleerup, Wanda K O'Neal, Richard Paine, Stephen P Peters, Neil E Alexis, Prescott G Woodruff, MeiLan K Han, Deborah A Meyers, Eugene R Bleecker
BACKGROUND: Increased concentrations of eosinophils in blood and sputum in chronic obstructive pulmonary disease (COPD) have been associated with increased frequency of exacerbations, reduced lung function, and corticosteroid responsiveness. We aimed to assess whether high eosinophil concentrations in either sputum or blood are associated with a severe COPD phenotype, including greater exacerbation frequency, and whether blood eosinophils are predictive of sputum eosinophils. METHODS: We did a multicentre observational study analysing comprehensive baseline data from SPIROMICS in patients with COPD aged 40-80 years who had a smoking history of at least 20 pack-years, recruited from six clinical sites and additional subsites in the USA between Nov 12, 2010, and April 21, 2015...
November 13, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29143692/non-pharmacological-interventions-for-lewy-body-dementia-a-systematic-review
#13
Michael H Connors, Lena Quinto, Ian McKeith, Henry Brodaty, Louise Allan, Claire Bamford, Alan Thomas, John-Paul Taylor, John T O'Brien
Lewy body dementia (consisting of dementia with Lewy bodies and Parkinson's disease dementia) is a common neurodegenerative disease characterised by visual hallucinations, fluctuating attention, motor disturbances, falls, and sensitivity to antipsychotics. This combination of features presents challenges for pharmacological management. Given this, we sought to review evidence for non-pharmacological interventions with patients with Lewy body dementia and their carers. Bibliographic databases were searched using a wide range of search terms and no restrictions were placed on study design, language, or clinical setting...
November 16, 2017: Psychological Medicine
https://www.readbyqxmd.com/read/29142760/outcomes-of-primary-endoresection-for-choroidal-melanoma
#14
Andre A C Vidoris, Andre Maia, Marcia Lowen, Melina Morales, Jordan Isenberg, Bruno F Fernandes, Rubens N Belfort
Purpose: To review long time treatment results in patients with small or medium sized choroidal melanoma who underwent surgical tumor endoresection as a primary treatment when plaque radiotherapy was unable and patients declined enucleation. Materials and methods: Patients were evaluated for best corrected visual acuity (BCVA), and underwent biomicroscopy, indirect ophthalmoscopy, retinography and ultrasound as well as the usual systemic workup. Study inclusion required the absence of scleral invasion or metastasis and an anterior margin not exceeding the pars plana or the ciliary body...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29142303/fus-toxicity-is-rescued-by-the-modulation-of-lncrna-hsr%C3%AF-expression-in-drosophila-melanogaster
#15
Luca Lo Piccolo, Salinee Jantrapirom, Yoshitaka Nagai, Masamitsu Yamaguchi
FUS is an aggregation-prone hnRNP involved in transcriptional and post-transcriptional regulation that aberrantly forms immunoreactive inclusion bodies in a range of neurological diseases classified as FUS-proteinopathies. Although FUS has been extensively examined, the underlying molecular mechanisms of these diseases have not yet been elucidated in detail. We previously reported that RNAi of the lncRNA hsrω altered the expression and sub-cellular localization of Drosophila FUS in the central nervous system of the fly...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29138281/a-conserved-cytoskeletal-signaling-cascade-mediates-neurotoxicity-of-ftdp-17-tau-mutations-in-vivo
#16
Farah H Bardai, Liqun Wang, Yamini Mutreja, Mythili Yenjerla, T Chris Gamblin, Mel B Feany
The microtubule binding protein tau is strongly implicated in multiple neurodegenerative disorders, including frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17), which is caused by mutations in tau. In vitro, FTDP-17 mutant versions of tau can reduce microtubule binding and increase aggregation of tau, but the mechanism by which these mutations promote disease in vivo is not clear. Here we take a combined biochemical and in vivo modeling approach to define functional properties of tau driving neurotoxicity in vivo We express wild type human tau and five FTDP-17 mutant forms of tau in Drosophila using a site-directed insertion strategy to ensure equivalent levels of expression...
November 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29131289/negative-predictors-of-treatment-success-in-outpatient-therapy-of-arterial-hypertension-in-poland-results-of-the-control-nt-observational-registry
#17
Krzysztof J Filipiak, Mariusz Tomaniak, Anna E Płatek, Filip M Szymański, Andrzej Tykarski
BACKGROUND: Identification of factors interfering with adequate control of arterial hypertension in the course of antihypertensive therapy is necessary to plan the strategy or reducing the incidence of cardiovascular disorders and optimize clinical practice guidelines. There have only been limited reliable studies describing the current status of hypertension treatment. AIM: The study objective was to conduct a screening assessment of the rate of uncontrolled hypertension among hypertensive patients coming to a routine follow-up visit, and to characterize patients with inadequate control of arterial hypertension in the aspect of their cardiovascular risk profile and therapeutic strategies used...
November 13, 2017: Kardiologia Polska
https://www.readbyqxmd.com/read/29131108/genetic-and-pathological-assessment-of-hnrnpa1-hnrnpa2-b1-and-hnrnpa3-in-familial-and-sporadic-amyotrophic-lateral-sclerosis
#18
Jennifer A Fifita, Katharine Y Zhang, Jasmin Galper, Kelly L Williams, Emily P McCann, Alison L Hogan, Neil Saunders, Denis Bauer, Ingrid S Tarr, Roger Pamphlett, Garth A Nicholson, Dominic Rowe, Shu Yang, Ian P Blair
BACKGROUND: Mutations in the genes encoding the heterogeneous nuclear ribonucleoproteins hnRNPA1 and hnRNPA2/B1 have been reported in a multisystem proteinopathy that includes amyotrophic lateral sclerosis (ALS) and inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia. Mutations were also described in the prion-like domain of hnRNPA1 in patients with classic ALS. Another hnRNP protein, hnRNPA3, has been found to be associated with the ALS/frontotemporal dementia protein C9orf72...
November 11, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/29127734/population-representation-among-anatomical-donors-and-the-implication-for-medical-student-education
#19
Amanda J Collins, Whitney Smith, Eustathia Lela Giannaris, Elizabeth Orvek, Peter Lazar, Jan K Carney, Anne M Gilroy, Max P Rosen
INTRODUCTION: Dissection provides a unique opportunity to integrate anatomical and clinical education. Commonly, cadavers are randomly assigned to courses, which may result in skewed representation of patient populations. The primary aim of this study was to determine if the anatomical donors studied by students at Medical School (MS) accurately represent the disease burden of the local patient population. MATERIALS AND METHODS: This cross-sectional study compared Medical Center patient claims data and body donation data from the Anatomical Gift Program (AGP)...
November 11, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/29127544/a-brazilian-family-with-inclusion-body-myopathy-associated-with-paget-s-disease-of-bone-and-frontotemporal-dementia-linked-to-the-vcp-pgly97glu-mutation
#20
REVIEW
Samuel Katsuyuki Shinjo, Sueli Mieko Oba-Shinjo, Antonio Marcondes Lerario, Suely Kazue Nagahashi Marie
The objective of this study is to report a Brazilian patient and his family with inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD). A systematic review of the literature on the valosin-containing protein (VCP) mutation was also performed. The proband (patient) was initially treated as a case of possible refractory polymyositis with Paget's disease and later as an inclusion body myopathy. However, after admission to our service, and considering his personal and familial antecedents, whole exome sequencing was performed revealing valosin-containing protein (VCP) c...
November 10, 2017: Clinical Rheumatology
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