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https://www.readbyqxmd.com/read/29904501/transpsoas-cystolitholapaxy-in-a-pediatric-patient-with-myelomeningocele-augmented-neobladder-and-mitrofanoff-appendicovesicostomy
#1
Spencer Lewis, Rajiv N Srinivasa, Jeffrey Forris Beecham Chick, Kate H Kraft, Casey A Dauw, Ravi N Srinivasa
Recurrent urolithiasis is a common and potentially morbid complication of spina bifida. Although the size, the location, and the composition of these stones often necessitates percutaneous intervention, access is often complicated by body habitus and spinal deformity. There is little consensus regarding the approach to percutaneous lithotripsy when a clear path to the collecting system is unavailable, particularly in the setting of surgically augmented urological anatomy. Here we present the first known case in which a pediatric patient with spina bifida underwent percutaneous lithotripsy of bladder stones via a transpsoas approach...
April 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29903984/bolus-ingestion-of-whey-protein-immediately-post-exercise-does-not-influence-rehydration-compared-to-energy-matched-carbohydrate-ingestion
#2
Gethin H Evans, Lewis Mattin, Isabelle Ireland, William Harrison, Adora M W Yau, Victoria McIver, Tristan Pocock, Elizabeth Sheader, Lewis J James
Whey protein is a commonly ingested nutritional supplement amongst athletes and regular exercisers; however, its role in post-exercise rehydration remains unclear. Eight healthy male and female participants completed two experimental trials involving the ingestion of 35 g of whey protein (WP) or maltodextrin (MD) at the onset of a rehydration period, followed by ingestion of water to a volume equivalent to 150% of the amount of body mass lost during exercise in the heat. The gastric emptying rates of the solutions were measured using 13 C breath tests...
June 14, 2018: Nutrients
https://www.readbyqxmd.com/read/29903629/magnetic-resonance-imaging-in-dementia
#3
L Raposo Rodríguez, D J Tovar Salazar, N Fernández García, L Pastor Hernández, Ó Fernández Guinea
OBJECTIVE: To describe and illustrate the key findings on structural magnetic resonance imaging (MRI) in the most common dementias of neurodegenerative origin: Alzheimer's disease, vascular dementia, dementia with Lewy bodies, variants of frontotemporal dementia, progressive supranuclear palsy, variants of multiple system atrophy, Parkinson dementia, and corticobasal degeneration. CONCLUSION: Today the role of MRI is no longer limited to ruling out underlying causes of cognitive deterioration...
June 11, 2018: Radiología
https://www.readbyqxmd.com/read/29903014/lrrk-2-gene-mutations-in-the-pathophysiology-of-the-roco-domain-and-therapeutic-targets-for-parkinson-s-disease-a-review
#4
REVIEW
Meng-Ling Chen, Ruey-Meei Wu
Parkinson's disease (PD) is the most common movement disorder and manifests as resting tremor, rigidity, bradykinesia, and postural instability. Pathologically, PD is characterized by selective loss of dopaminergic neurons in the substantia nigra and the formation of intracellular inclusions containing α-synuclein and ubiquitin called Lewy bodies. Consequently, a remarkable deficiency of dopamine in the striatum causes progressive disability of motor function. The etiology of PD remains uncertain. Genetic variability in leucine-rich repeat kinase 2 (LRRK2) is the most common genetic cause of sporadic and familial PD...
June 14, 2018: Journal of Biomedical Science
https://www.readbyqxmd.com/read/29899523/relation-of-placental-alkaline-phosphatase-expression-in-human-term-placenta-with-maternal-and-offspring-fat-mass
#5
Birgit Hirschmugl, Sarah Crozier, Nina Matthews, Eva Kitzinger, Ingeborg Klymiuk, Hazel M Inskip, Nicholas C Harvey, Cyrus Cooper, Colin P Sibley, Jocelyn Glazier, Christian Wadsack, Keith M Godfrey, Gernot Desoye, Rohan M Lewis
INTRODUCTION: Alkaline phosphatase is implicated in intestinal lipid transport and in the development of obesity. Placental alkaline phosphatase is localised to the microvillous plasma membrane of the placental syncytiotrophoblast at the maternal-fetal interface, but its role is unclear. We investigated the relations of placental alkaline phosphatase activity and mRNA expression with maternal body composition and offspring fat mass in humans. METHODS: Term human placentas from the UK Birthright cohort (n = 52) and the Southampton Women's Survey (SWS) (n = 95) were studied...
June 13, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29899472/imaging-egfr-and-her3-through-89-zr-labeled-mehd7945a-duligotuzumab
#6
Brooke N McKnight, Akhila N W Kuda-Wedagedara, Kuntal K Sevak, Dalya Abdel-Atti, Wendy N Wiesend, Anson Ku, Dakshnamurthy Selvakumar, Sean D Carlin, Jason S Lewis, Nerissa T Viola-Villegas
Tumor resistance to treatment paved the way toward the development of single agent drugs that target multiple molecular signatures amplified within the malignancy. The discovered crosstalk between EGFR and HER3 as well as the role of HER3 in mediating EGFR resistance made these two receptor tyrosine kinases attractive targets. MEHD7945A or duligotuzumab is a single immunotherapy agent that dually targets both molecular signatures. In this study, a positron emission tomography (PET) companion diagnostic to MEHD7945A is reported and evaluated in pancreatic cancer...
June 13, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29898972/diffuse-lewy-body-disease-manifesting-as-corticobasal-syndrome-a-rare-form-of-lewy-body-disease
#7
Koji Kasanuki, Keith A Josephs, Tanis J Ferman, Melissa E Murray, Shunsuke Koga, Takuya Konno, Nobutaka Sakae, Adam Parks, Ryan J Uitti, Jay A Van Gerpen, Neill R Graff-Radford, Zbigniew K Wszolek, Dennis W Dickson
OBJECTIVE: To describe clinical and pathologic characteristics of diffuse Lewy body disease (DLBD) manifesting as corticobasal syndrome (CBS). METHODS: In 523 autopsy-confirmed cases of DLBD, we identified 11 patients diagnosed with CBS. For comparison, we studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). Given previous studies suggesting the importance of pathology in peri-Rolandic cortices in CBS, we used digital pathology to count Lewy bodies and to quantify intracytoplasmic and neuritic α-synuclein and phospho-tau burden in the motor cortex...
June 13, 2018: Neurology
https://www.readbyqxmd.com/read/29898700/development-and-testing-of-the-reliability-and-validity-of-the-adolescent-haze-related-knowledge-awareness-assessment-scale-ahrkaas
#8
Hongzhe Dou, Yuejia Zhao, Yanhong Chen, Qingchun Zhao, Bo Xiao, Yan Wang, Yonghe Zhang, Zhiguo Chen, Jie Guo, Lingwei Tao
BACKGROUND: Haze leads to many direct serious public health impacts. Understanding haze related knowledge can not only help adolescents organize health protection awareness to prevent the harmful effects that haze has on the body, but also promote their normal growth and development. METHODS: By considering, as the theoretical basis, the reasons behind the formation of haze and the underlying mechanisms of the diseases that it causes, in addition to also investigating extensive literature references, our research team developed the Adolescent Haze Related Knowledge Awareness Assessment Scale (AHRKAAS-I)...
June 14, 2018: BMC Public Health
https://www.readbyqxmd.com/read/29898690/a-comparison-of-temporal-artery-thermometers-with-internal-blood-monitors-to-measure-body-temperature-during-hemodialysis
#9
Meaghan Lunney, Bronwyn Tonelli, Rachel Lewis, Natasha Wiebe, Chandra Thomas, Jennifer MacRae, Marcello Tonelli
BACKGROUND: Thermometers that measure core (internal) body temperature are the gold standard for monitoring temperature. Despite that most modern hemodialysis machines are equipped with an internal blood monitor that measures core body temperature, current practice is to use peripheral thermometers. A better understanding of how peripheral thermometers compare with the dialysis machine thermometer may help guide practice. METHODS: The study followed a prospective cross-sectional design...
June 14, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29897434/the-c-abl-inhibitor-radotinib-hcl-is-neuroprotective-in-a-preclinical-parkinson-s-disease-mouse-model
#10
Saebom Lee, Sangjune Kim, Yong Joo Park, Seung Pil Yun, Seung-Hwan Kwon, Donghoon Kim, Dong Yeon Kim, Jae Soo Shin, Dae Jin Cho, Gong Yeal Lee, Hyun Soo Ju, Hyo Jung Yun, Jae Hong Park, Wonjoong Richard Kim, Eun Ah Jung, Seulki Lee, Han Seok Ko
Accumulating evidence suggests that the nonreceptor tyrosine kinase c-Abl plays an important role in the progression of Parkinson's disease (PD) and c-Abl inhibition could be neuroprotective in PD and related α-synucleinopathies. Nilotinib, a c-Abl inhibitor, has shown improved motor and cognitive symptoms in PD patients. However, issues concerning blood-brain barrier (BBB) penetration, lack of selectivity, and safety still remain. Radotinib HCl is a selective Bcr-Abl kinase inhibitor that not only effectively access the brain, but also exhibits greater pharmacokinetic properties and safety profiles compared to Nilotinib and other c-Abl inhibitors...
April 20, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29896411/glucocerebrosidase-mrna-is-diminished-in-brain-of-lewy-body-diseases-and-changes-with-disease-progression-in-blood
#11
Laia Perez-Roca, Cristina Adame-Castillo, Jaume Campdelacreu, Lourdes Ispierto, Dolores Vilas, Ramon Rene, Ramiro Alvarez, Jordi Gascon-Bayarri, Maria A Serrano-Munoz, Aurelio Ariza, Katrin Beyer
Parkinson disease (PD) and dementia with Lewy bodies (DLB) are Lewy body diseases characterized by abnormal alpha-synuclein deposits and overlapping pathological features in the brain. Several studies have shown that glucocerebrosidase (GBA) deficiency is involved in the development of LB diseases. Here, we aimed to find out if this deficiency starts at the transcriptional level, also involves alternative splicing, and if GBA expression changes in brain are also detectable in blood of patients with LB diseases...
April 2018: Aging and Disease
https://www.readbyqxmd.com/read/29892352/effects-of-exposure-to-bodies-of-different-sizes-on-perception-of-and-satisfaction-with-own-body-size-two-randomized-studies
#12
Helen Bould, Rebecca Carnegie, Heather Allward, Emily Bacon, Emily Lambe, Megan Sapseid, Katherine S Button, Glyn Lewis, Andy Skinner, Matthew R Broome, Rebecca Park, Catherine J Harmer, Ian S Penton-Voak, Marcus R Munafò
Body dissatisfaction is prevalent among women and associated with subsequent obesity and eating disorders. Exposure to images of bodies of different sizes has been suggested to change the perception of 'normal' body size in others. We tested whether exposure to different-sized (otherwise identical) bodies changes perception of own and others' body size, satisfaction with body size and amount of chocolate consumed. In Study 1, 90 18-25-year-old women with normal BMI were randomized into one of three groups to complete a 15 min two-back task using photographs of women either of 'normal weight' (Body Mass Index (BMI) 22-23 kg m-2 ), or altered to appear either under- or over-weight...
May 2018: Royal Society Open Science
https://www.readbyqxmd.com/read/29889265/systematic-screening-of-ubiquitin-p62-aggregates-in-cerebellar-cortex-expands-the-neuropathological-phenotype-of-the-c9orf72-expansion-mutation
#13
Oscar Ramos-Campoy, Rainiero Ávila-Polo, Oriol Grau-Rivera, Anna Antonell, Jordi Clarimón, Ricardo Rojas-García, Sara Charif, Veronica Santiago-Valera, Isabel Hernandez, Miquel Aguilar, Consuelo Almenar, Dolores Lopez-Villegas, Lorena Bajo, Pau Pastor, Julie Van der Zee, Albert Lladó, Raquel Sanchez-Valle, Ellen Gelpi
The neuropathological hallmark of the C9orf72 intronic hexanucleotide expansion in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) is the presence of small ubiquitin/p62-positive and transactive response DNA binding protein 43 kDa (TDP-43)-negative cytoplasmic inclusions in several brain areas. The identification of this histopathological signature is highly predictive of an underlying mutation. In this study, we screened 1800 cases of the Barcelona IDIBAPS Brain Bank, independently of the clinical and final neuropathological diagnosis of the brain donor, for the presence of ubiquitin/p62-positive inclusions in the cerebellum (UPPI)...
June 7, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29889064/clinical-subtypes-of-dementia-with-lewy-bodies-based-on-the-initial-clinical-presentation
#14
Estrella Morenas-Rodríguez, Isabel Sala, Andrea Subirana, Elba Pascual-Goñi, MaBelén Sánchez-Saudinós, Daniel Alcolea, Ignacio Illán-Gala, María Carmona-Iragui, Roser Ribosa-Nogué, Valle Camacho, Rafael Blesa, Juan Fortea, Alberto Lleó
BACKGROUND: Dementia with Lewy bodies (DLB) is a heterogeneous disease in which clinical presentation, symptoms, and evolution widely varies between patients. OBJECTIVE: To investigate the existence of clinical subtypes in DLB based on the initial clinical presentation. METHODS: 81 patients with a clinical diagnosis of probable DLB were consecutively included. All patients underwent a neurological evaluation including a structured questionnaire about neuropsychiatric symptoms and sleep, an assessment of motor impairment (Unified Parkinson Disease Rating Scale subscale III), and a formal neuropsychological evaluation...
June 4, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29888794/sensitivity-and-specificity-of-phospho-ser129-%C3%AE-synuclein-monoclonal-antibodies
#15
Vedad Delic, Sidhanth Chandra, Hisham Abdelmotilib, Tyler Maltbie, Shijie Wang, Danielle Kem, Hunter J Scott, Rachel N Underwood, Zhiyong Liu, Laura A Volpicelli-Daley, Andrew B West
α-Synuclein (α-syn) is an abundant presynaptic protein that is the primary constituent of inclusions that define Lewy body diseases (LBDs). In these inclusions, α-syn is phosphorylated at the serine-129 residue. Antibodies directed to this phosphorylation site are used to measure inclusion abundance and stage disease progression in pre-clinical models as well as in post-mortem tissues in LBDs. While it is critical to reliably identify inclusions, phospho-specific antibodies often cross-react with non-specific antigens...
June 11, 2018: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/29888220/exploring-the-structural-diversity-in-inhibitors-of-%C3%AE-synuclein-amyloidogenic-folding-aggregation-and-neurotoxicity
#16
Sukanya Das, Tara L Pukala, Scott D Smid
Aggregation of α-Synuclein (αS) protein to amyloid fibrils is a neuropathological hallmark of Parkinson's disease (PD). Growing evidence suggests that extracellular αS aggregation plays a pivotal role in neurodegeneration found in PD in addition to the intracellular αS aggregates in Lewy bodies (LB). Here, we identified and compared a diverse set of molecules capable of mitigating protein aggregation and exogenous toxicity of αSA53T, a more aggregation-prone αS mutant found in familial PD. For the first time, we investigated the αS anti-amyloid activity of semi-synthetic flavonoid 2', 3', 4' trihydroxyflavone or 2-D08, which was compared with natural flavones myricetin and transilitin, as well as such structurally diverse polyphenols as honokiol and punicalagin...
2018: Frontiers in Chemistry
https://www.readbyqxmd.com/read/29887357/dnajc13-mutation-screening-in-patients-with-parkinson-s-disease-from-south-italy
#17
Monica Gagliardi, Grazia Annesi, Radha Procopio, Maurizio Morelli, Grazia Iannello, Giuseppe Bonapace, Manuela Mancini, Giuseppe Nicoletti, Aldo Quattrone
BACKGROUND: Parkinson's disease (PD) is the second most common neurodegenerative disorder, and the most common neurodegenerative form of parkinsonism. Recently, a pathogenic mutation (p.N855S) in DNAJC13 was linked to autosomal dominant Lewy body PD in a Dutch-German-Russian Mennonite multi-incident kindred, and was found in five additional patients. In this study, we performed a comprehensive screening of the DNAJC13 gene in familial PD and sporadic PD to assess the frequency of known and novel rare nonsynonymous variants...
June 4, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29887161/lrp10-genetic-variants-in-familial-parkinson-s-disease-and-dementia-with-lewy-bodies-a-genome-wide-linkage-and-sequencing-study
#18
Marialuisa Quadri, Wim Mandemakers, Martyna M Grochowska, Roy Masius, Hanneke Geut, Edito Fabrizio, Guido J Breedveld, Demy Kuipers, Michelle Minneboo, Leonie J M Vergouw, Ana Carreras Mascaro, Ekaterina Yonova-Doing, Erik Simons, Tianna Zhao, Alessio B Di Fonzo, Hsiu-Chen Chang, Piero Parchi, Marta Melis, Leonor Correia Guedes, Chiara Criscuolo, Astrid Thomas, Rutger W W Brouwer, Daphne Heijsman, Angela M T Ingrassia, Giovanna Calandra Buonaura, Janneke P Rood, Sabina Capellari, Annemieke J Rozemuller, Marianna Sarchioto, Hsin Fen Chien, Nicola Vanacore, Simone Olgiati, Yah-Huei Wu-Chou, Tu-Hsueh Yeh, Agnita J W Boon, Susanne E Hoogers, Mehrnaz Ghazvini, Arne S IJpma, Wilfred F J van IJcken, Marco Onofrj, Paolo Barone, David J Nicholl, Andreas Puschmann, Michele De Mari, Anneke J Kievit, Egberto Barbosa, Giuseppe De Michele, Danielle Majoor-Krakauer, John C van Swieten, Frank J de Jong, Joaquim J Ferreira, Giovanni Cossu, Chin-Song Lu, Giuseppe Meco, Pietro Cortelli, Wilma D J van de Berg, Vincenzo Bonifati
BACKGROUND: Most patients with Parkinson's disease, Parkinson's disease dementia, and dementia with Lewy bodies do not carry mutations in known disease-causing genes. The aim of this study was to identify a novel gene implicated in the development of these disorders. METHODS: Our study was done in three stages. First, we did genome-wide linkage analysis of an Italian family with dominantly inherited Parkinson's disease to identify the disease locus. Second, we sequenced the candidate gene in an international multicentre series of unrelated probands who were diagnosed either clinically or pathologically with Parkinson's disease, Parkinson's disease dementia, or dementia with Lewy bodies...
June 6, 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29887160/a-novel-link-between-trafficking-and-lewy-body-disorders
#19
Owen A Ross, Guojun Bu
No abstract text is available yet for this article.
June 6, 2018: Lancet Neurology
https://www.readbyqxmd.com/read/29887143/prion-like-propagation-of-pathology-in-parkinson-disease
#20
Laura Volpicelli-Daley, Patrik Brundin
Over 100 years ago, Lewy bodies and Lewy neurites were defined as a pathologic hallmark of Parkinson disease. Eighty years later, α-synuclein was found to be the primary component of these inclusions. Emerging evidence suggests that α-synuclein pathology propagates across interconnected networks throughout the nervous system in a prion-like manner. Pathologic α-synuclein seeds aggregation of native α-synuclein, resulting in the formation of insoluble inclusions. These seeds can propagate within the neuron and to interconnected neurons, resulting in the spread of pathology throughout the brain...
2018: Handbook of Clinical Neurology
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