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Dementia With Lewy Bodies

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RT-QuIC-based detection of alpha-synuclein seeding activity in brains of dementia with Lewy Body patients and of a transgenic mouse model of synucleinopathy.

Jung-Youn Han, Hyung-Sup Jang, Alison J E Green, Young Pyo Choi,

RT-QuIC is a shaking-based cyclic amplification technique originally developed in the prion field to detect minute amounts of scrapie prion protein (PrPSc). In this study, we applied the RT-QuIC assay to investigate a-synuclein (a-syn) seeding activity in brains of Dementia with Lewy Body (DLB) patients and in brains of G2-3 ... Read more >>

Prion (Prion)
[2020, 14(1):88-94]

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The roles of atypical protein kinase Cs (aPKCs) in the nervous system: targets for neuroregeneration?

Dennis Dik-Long Chau, Kwok-Fai Lau,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1475-1476]

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Rifampicin quinone pretreatment improves neuronal survival by modulating microglia inflammation induced by α-synuclein.

Leonardo Acuna, Natalia S Corbalán, Rita Raisman-Vozari,

Neural Regen Res (Neural regeneration research)
[2020, 15(8):1473-1474]

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Clinical and pathological features affecting cardiac sympathetic denervation in autopsy-confirmed dementia with Lewy bodies.

Makoto Takahashi, Toshiki Uchihara, Mari Yoshida, Koichi Wakabayashi, Akiyoshi Kakita, Hitoshi Takahashi, Shuta Toru, Satoshi Orimo,

BACKGROUND:To clarify the features affecting cardiac sympathetic denervation in autopsy-confirmed dementia with Lewy bodies (DLB) patients. METHODS:Fifty-four autopsy-confirmed DLB patients were enrolled. Tissue samples of the left ventricular anterior wall were immunostained with anti-tyrosine hydroxylase antibody to identify catecholaminergic nerve axons. We quantified immunostained areas as residual cardiac sympathetic nerve ... Read more >>

Eur. J. Neurol. (European journal of neurology)
[2020, :]

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Cerebrospinal fluid neurogranin as a new player in prion disease diagnosis and prognosis.

Anna Villar-Pique, Inga Zerr, Franc Llorens,

Neural Regen Res (Neural regeneration research)
[2020, 15(5):861-862]

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Clinicopathologic and genetic features of multiple system atrophy with Lewy body disease.

Shunsuke Koga, Fuyao Li, Na Zhao, Shanu F Roemer, Tanis J Ferman, Anna I Wernick, Ronald L Walton, Ayman H Faroqi, Neill R Graff-Radford, William P Cheshire, Owen A Ross, Dennis W Dickson,

BACKGROUND:Abnormal aggregates of α-synuclein are pathologic hallmarks of multiple system atrophy (MSA) and Lewy body disease (LBD). LBD sometimes coexists with MSA, but the impact of co-pathology, particularly diffuse LBD, on presentation of MSA has not been studied. We aimed to determine the frequency and clinicopathologic features of MSA with ... Read more >>

Brain Pathol. (Brain pathology (Zurich, Switzerland))
[2020, :]

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FDG Uptake in the Basal Forebrain as Measured by Digital High-Resolution PET Is a Promising Marker of Basal Forebrain Degeneration in the Lewy Body Disease Spectrum: A Pilot Study.

Cansu Özden, Lars Frings, Ivayla Apostolova, Catharina Lange, Susanne Klutmann, Gerhard Adam, Peter Bannas, Philipp T Meyer, Michel J Grothe, Ralph Buchert,

PURPOSE:Cognitive decline in diseases of the Lewy body spectrum (LBS) is linked to dysfunction/degeneration of the basal forebrain (BF). Assessment of glucose metabolism in the BF by FDG PET is hampered by the small size of the BF and limited spatial resolution of conventional PET. This pilot study tested the ... Read more >>

Clin Nucl Med (Clinical nuclear medicine)
[2020, 45(4):261-266]

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[Urinary tract symptoms in Lewy body dementia: About 19 cases].

V Coindreau, C Chesnel, F Babany, A Declemy, E Savard, A Charlanes, F Lebreton, G Amarenco,

INTRODUCTION:Lewy Body Dementia (LBD) is a Parkinsonian disorder which often leads to Lower Urinary Tract Symptoms (LUTS), especially an Overactive Bladder (OAB). There have been few LBD related LUTS depictions in the literature, which is why we did this retrospective study. METHODS:Retrospective single institution study. RESULTS:Nineteen patients with confirmed LBD ... Read more >>

Prog. Urol. (Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologie)
[2020, :]

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Fabry Disease With Concomitant Lewy Body Disease.

Kelly Del Tredici, Albert C Ludolph, Simone Feldengut, Christian Jacob, Heinz Reichmann, Jürgen R Bohl, Heiko Braak,

Although Gaucher disease can be accompanied by Lewy pathology (LP) and extrapyramidal symptoms, it is unknown if LP exists in Fabry disease (FD), another progressive multisystem lysosomal storage disorder. We aimed to elucidate the distribution patterns of FD-related inclusions and LP in the brain of a 58-year-old cognitively unimpaired male ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):378-392]

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α-Synuclein heteromers with ß-amyloid and tau decreased in red blood cells of Alzheimer’s disease and Lewy Body dementia patients.

Claudia Martini, Simona Daniele, Filippo Baldacci, Rebecca Piccarducci, Giovanni Palermo, Linda Giampietri, Laura Manca, Deboarh Pietrobono, Daniela Frosini, Valentina Nicoletti, Gloria Tognoni, Filippo Giorgi, Annalisa Gerfo, Lucia Petrozzi, Chiara Cavallini, Ferdinando Franzoni, Roberto Ceravolo, Gabriele Siciliano, Maria Trincavelli, Ubaldo Bonuccelli,

<title>Abstract</title> <p> Background Red blood cells (RBC) account for more than 99% of α-syn concentrations in blood representing an interesting in vivo model for studying peripheral pathological alterations proved in neurodegeneration. The aim of the current study was to investigate the diagnostic value of total α-syn, Aβ 1-42 ... Read more >>

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Generation and Characterization of Novel Monoclonal Antibodies Targeting p62/sequestosome-1 Across Human Neurodegenerative Diseases.

Jorge A Trejo-Lopez, Zachary A Sorrentino, Cara J Riffe, Stefan Prokop, Dennis W Dickson, Anthony T Yachnis, Benoit I Giasson,

Human neurodegenerative diseases can be characterized as disorders of protein aggregation. As a key player in cellular autophagy and the ubiquitin proteasome system, p62 may represent an effective immunohistochemical target, as well as mechanistic operator, across neurodegenerative proteinopathies. In this study, 2 novel mouse-derived monoclonal antibodies 5G3 and 2A5 raised ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):407-418]

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[Autonomic dysfunction in the patient with neurodegenerative disease].

R Roa-Chamorro, L Torres-Quintero, P González-Bustos,

Autonomic dysfunction is a common condition in the alpha-synucleinopathies (Parkinson's disease, dementia with Lewy bodies, multiple system atrophy). Cardiovascular symptoms may include orthostatic hypotension, supine hypertension or decreased heart rate response. A clinical suspicion and physical examination are essential for diagnosis, taking blood pressure in supine and standing positions. The ... Read more >>

Hipertens Riesgo Vasc (Hipertension y riesgo vascular)
[2020, :]

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Confirmation of 123I-FP-CIT-SPECT (ioflupane) quantification methods in dementia with Lewy body and other neurodegenerative disorders.

Daniela D Maltais, Lennon G Jordan, Hoon-Ki Min, Toji Miyawaga, Scott Pryzbleski, Timothy G Lesnick, Robert Ross Reichard, Dennis W Dickson, Melissa E Murray, Kejal Kantarci, Bradley F Boeve, Val Lowe,

Rationale: To conduct a retrospective study comparing three 123I-FP-CIT-SPECT quantitative methods in patients with neurodegenerative syndromes as referenced to neuropathological findings. Methods: 123I-FP-CIT-SPECT and neuropathological findings among patients with neurodegenerative syndromes from the Mayo Alzheimer's Disease Research Center and Mayo Clinic Study of Aging were examined. Three 123I-FP-CIT-SPECT quantitative assessment ... Read more >>

J. Nucl. Med. (Journal of nuclear medicine : official publication, Society of Nuclear Medicine)
[2020, :]

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Systematic review of gene expression studies in people with Lewy Body Dementia.

Anisa Chowdhury, Anto P Rajkumar,

OBJECTIVES:Lewy body dementia (LBD) is the second most prevalent neurodegenerative dementia, and it causes more morbidity and mortality than Alzheimer's disease. Several genetic associations of LBD have been reported, and their functional implications remain uncertain. Hence, we aimed to do a systematic review of all gene expression studies that investigated ... Read more >>

Acta Neuropsychiatr (Acta neuropsychiatrica)
[2020, :1-42]

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Total alpha-synuclein assay in the Cerebro-Spinal-Fluid is of interest in the differential diagnosis between Alzheimer's disease and dementia with Lewy bodies from the prodromal stage

Olivier BOUSIGES, Nathalie Philippi, Thomas Lavaux, Armand Perret-Liaudet, Ingolf Lachmann, Caroline Schaeffer-Agalède, Pierre Anthony, Anne Botzung, Lucie Rauch, Barbara Jung, Paulo Sousa, Catherine Demuynck, Catherine Martin-Hunyadi, Benjamin Cretin, Frédéric Blanc,

<title>Abstract</title> <p> Background: Several studies have investigated the value of alpha-synuclein assay in the cerebrospinal fluid (CSF) of Alzheimer’s disease (AD) and Dementia with Lewy Bodies (DLB) patients in the differential diagnosis of these two pathologies. However, very few studies have focused on this assay in AD and ... Read more >>

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Automated voxel- and region-based analysis of gray matter and cerebrospinal fluid space in primary dementia disorders.

Karl Egger, Alexander Rau, Shan Yang, Stefan Klöppel, Ahmed Abdulkadir, Elias Kellner, Lars Frings, Sabine Hellwig, Horst Urbach, ,

PURPOSE:Previous studies showed voxel-based volumetry as a helpful tool in detecting pathologic brain atrophy. Aim of this study was to investigate whether the inclusion of CSF volume improves the imaging based diagnostic accuracy by using combined automated voxel- and region-based volumetry. METHODS:In total, 120 individuals (30 healthy elderly, 30 frontotemporal ... Read more >>

Brain Res. (Brain research)
[2020, :146800]

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Diagnosing multiple system atrophy at the prodromal stage.

Chenjie Xia, Ronald B Postuma,

Identifying individuals at the earliest disease stage becomes crucial as we aim to develop disease-modifying treatments for neurodegenerative disorders. Prodromal diagnostic criteria were recently developed for Parkinson's disease (PD) and are forthcoming for dementia with Lewy bodies (DLB). The latest 2008 version of diagnostic criteria for multiple system atrophy (MSA) ... Read more >>

Clin. Auton. Res. (Clinical autonomic research : official journal of the Clinical Autonomic Research Society)
[2020, :]

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A Phase 2a Trial Investigating the Safety and Tolerability of the Novel Cortical Enhancer IRL752 in Parkinson's Disease Dementia.

Per Svenningsson, Per Odin, Nil Dizdar, Anders Johansson, Sotirios Grigoriou, Panagiota Tsitsi, Klas Wictorin, Filip Bergquist, Dag Nyholm, Juha Rinne, Fredrik Hansson, Clas Sonesson, Joakim Tedroff, ,

BACKGROUND:IRL752 is a novel small-molecule compound that acts to regioselectively enhance norepinephrine, dopamine, and acetylcholine neurotransmission in the cerebral cortex. OBJECTIVE:The primary objective of the trial was to investigate the safety and tolerability of IRL752 in patients with Parkinson's disease and dementia. METHODS:Patients with Parkinson's disease and dementia were randomized ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, :]

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Neuropathological correlates of parkinsonian disorders in a large Dutch autopsy series.

H Geut, D H Hepp, E Foncke, H W Berendse, J M Rozemuller, I Huitinga, W D J van de Berg,

The clinical diagnosis in patients with parkinsonian disorders can be challenging, and a definite diagnosis requires neuropathological confirmation. The aim of this study was to examine whether a clinical diagnosis of Parkinson's disease (PD) and atypical parkinsonian disorders predict the presence of Lewy pathology (LP) and concomitant neuropathological lesions.We included ... Read more >>

Acta Neuropathol Commun (Acta neuropathologica communications)
[2020, 8(1):39]

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Autonomic dysfunction is associated with neuropsychological impairment in Lewy body disease.

Rocío Del Pino, Ane Murueta-Goyena, Marian Acera, Mar Carmona-Abellan, Beatriz Tijero, Olaia Lucas-Jiménez, Natalia Ojeda, Naroa Ibarretxe-Bilbao, Javier Peña, Iñigo Gabilondo, Juan Carlos Gómez-Esteban,

OBJECTIVE:This study aimed to analyze the association of autonomic dysfunction with cognition, depression, apathy, and fatigue in Lewy body disease (LBD). METHODS:We included 61 patients [49 with idiopathic Parkinson's disease, 7 with dementia with Lewy bodies, and 5 E46K-SNCA mutation carriers] and 22 healthy controls. All participants underwent a comprehensive ... Read more >>

J. Neurol. (Journal of neurology)
[2020, :]

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Spatial metabolic profiles to discriminate dementia with Lewy bodies from Alzheimer disease.

Tomomichi Iizuka, Masashi Kameyama,

BACKGROUND:To differentiate dementia with Lewy bodies (DLB) from Alzheimer disease (AD) using a single imaging modality is challenging, because of their common hypometabolic findings. Scaled subprofile modeling/principal component analysis (SSM/PCA), an unsupervised artificial intelligence, has the potential to offer an alternative to image analysis. OBJECTIVE:We aimed to produce spatial metabolic ... Read more >>

J. Neurol. (Journal of Neurology)
[2020, :]

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Fatty Acid Binding Protein 3 Enhances the Spreading and Toxicity of α-Synuclein in Mouse Brain.

Yasushi Yabuki, Kazuya Matsuo, Ichiro Kawahata, Naoya Fukui, Tomohiro Mizobata, Yasushi Kawata, Yuji Owada, Norifumi Shioda, Kohji Fukunaga,

Oligomerization and/or aggregation of α-synuclein (α-Syn) triggers α-synucleinopathies such as Parkinson's disease and dementia with Lewy bodies. It is known that α-Syn can spread in the brain like prions; however, the mechanism remains unclear. We demonstrated that fatty acid binding protein 3 (FABP3) promotes propagation of α-Syn in mouse brain. ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(6):]

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Higher vitamin B12 level at Parkinson's disease diagnosis is associated with lower risk of future dementia.

Stuart J McCarter, Cole Stang, Pierpaolo Turcano, Michelle M Mielke, Farwa Ali, James H Bower, Rodolfo Savica,

INTRODUCTION:To determine whether vitamin B12 level at Parkinson's disease (PD) diagnosis predicts time to develop dementia. METHODS:We utilized a population-based cohort of Parkinsonism patients to examine the relationship between serum vitamin B12 at the time of PD diagnosis and dementia risk. Receiver operating curves were calculated for vitamin B12 cutoffs ... Read more >>

Parkinsonism Relat. Disord. (Parkinsonism & related disorders)
[2020, 73:19-22]

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Role of Alcohol Drinking in Alzheimer's Disease, Parkinson's Disease, and Amyotrophic Lateral Sclerosis.

Bin Peng, Qiang Yang, Rachna B Joshi, Yuancai Liu, Mohammed Akbar, Byoung-Joon Song, Shuanhu Zhou, Xin Wang,

Neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD) and amyotrophic lateral sclerosis (ALS), increase as the population ages around the world. Environmental factors also play an important role in most cases. Alcohol consumption exists extensively and it acts as one of the environmental factors that promotes these neurodegenerative diseases. ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(7):]

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Visual hallucinations in neurological and ophthalmological disease: pathophysiology and management.

John O'Brien, John Paul Taylor, Clive Ballard, Roger A Barker, Clare Bradley, Alistair Burns, Daniel Collerton, Sonali Dave, Rob Dudley, Paul Francis, Andrea Gibbons, Kate Harris, Vanessa Lawrence, Iracema Leroi, Ian McKeith, Michel Michaelides, Chaitali Naik, Claire O'Callaghan, Kirsty Olsen, Marco Onofrj, Rebecca Pinto, Gregor Russell, Peter Swann, Alan Thomas, Prabitha Urwyler, Rimona Sharon Weil, Dominic Ffytche,

Visual hallucinations are common in older people and are especially associated with ophthalmological and neurological disorders, including dementia and Parkinson's disease. Uncertainties remain whether there is a single underlying mechanism for visual hallucinations or they have different disease-dependent causes. However, irrespective of mechanism, visual hallucinations are difficult to treat. The ... Read more >>

J. Neurol. Neurosurg. Psychiatry (Journal of neurology, neurosurgery, and psychiatry)
[2020, :]

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