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Lewy body disease

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https://www.readbyqxmd.com/read/29244218/person-specific-contribution-of-neuropathologies-to-cognitive-loss-in-old-age
#1
Patricia A Boyle, Lei Yu, Robert S Wilson, Sue E Leurgans, Julie A Schneider, David A Bennett
OBJECTIVE: Mixed neuropathologies are the most common cause of dementia at the population level, but how different neuropathologies contribute to cognitive decline at the individual level remains unknown. We quantified the contribution of nine neuropathologies to cognitive loss at an individual level. METHODS: Participants (n=1,079) came from 2 longitudinal clinical-pathologic studies of aging. All completed 2+ cognitive evaluations (maximum = 22), died and underwent neuropathologic examinations to identify Alzheimer's disease (AD), other neurodegenerative diseases, and vascular pathologies...
December 15, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29243911/neurodegenerative-disease-proteinopathies-are-connected-to-distinct-histone-post-translational-modification-landscapes
#2
Karen Chen, Seth A Bennett, Navin Rana, Huda Yousuf, Mohamed Said, Sadiqa Taaseen, Natalie Mendo, Steven Marc Meltser, Mariana Torrente
Amyotrophic Lateral Sclerosis (ALS) and Parkinson's disease (PD) are devastating neurodegenerative diseases involving the progressive degeneration of neurons. No cure is available for patients diagnosed with these diseases. A prominent feature for both ALS and PD is the accumulation of protein inclusions in the cytoplasm of degenerating neurons; however, the particular protein comprising these inclusions varies. The RNA-binding proteins TDP-43 and FUS are most notable in ALS, while α-synuclein aggregates into Lewy bodies in PD...
December 15, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29240340/update-on-current-care-guideline-memory-disorders
#3
(no author information available yet)
Any complaint about memory should be examined. Diagnosis is based on international criteria. Basic evaluation consists of medical history, clinical evaluation, cognitive tests and brain imaging. When a diagnosis of Alzheimer's disease (AD), AD with cerebrovascular disease or with Lewy Body disease (LBD), or Dementia associated with Parkinson's disease or LBD is made, evidence-based medical therapy (acetylcholine esterase inhibitor or memantine) is indicated as a part of comprehensive care. In frontotemporal degenerations, these drugs are ineffective...
2017: Duodecim; Lääketieteellinen Aikakauskirja
https://www.readbyqxmd.com/read/29236052/micrornas-in-parkinson-s-disease-from-pathogenesis-to-novel-diagnostic-and-therapeutic-approaches
#4
REVIEW
Loredana Leggio, Silvia Vivarelli, Francesca L'Episcopo, Cataldo Tirolo, Salvo Caniglia, Nunzio Testa, Bianca Marchetti, Nunzio Iraci
Parkinson's disease (PD) is the most prevalent central nervous system (CNS) movement disorder and the second most common neurodegenerative disease overall. PD is characterized by the progressive loss of dopaminergic (DAergic) neurons in the substantia nigra pars compacta (SNpc) within the midbrain, accumulation of alpha-synuclein (α-SYN) in Lewy bodies and neurites and excessive neuroinflammation. The neurodegenerative processes typically begin decades before the appearance of clinical symptoms. Therefore, the diagnosis is achievable only when the majority of the relevant DAergic neurons have already died and for that reason available treatments are only palliative at best...
December 13, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29233218/secondary-nucleation-of-monomers-on-fibril-surface-dominates-%C3%AE-synuclein-aggregation-and-provides-autocatalytic-amyloid-amplification
#5
Ricardo Gaspar, Georg Meisl, Alexander K Buell, Laurence Young, Clemens F Kaminski, Tuomas P J Knowles, Emma Sparr, Sara Linse
Parkinson's disease (PD) is characterized by proteinaceous aggregates named Lewy Bodies and Lewy Neurites containing α-synuclein fibrils. The underlying aggregation mechanism of this protein is dominated by a secondary process at mildly acidic pH, as in endosomes and other organelles. This effect manifests as a strong acceleration of the aggregation in the presence of seeds and a weak dependence of the aggregation rate on monomer concentration. The molecular mechanism underlying this process could be nucleation of monomers on fibril surfaces or fibril fragmentation...
January 2017: Quarterly Reviews of Biophysics
https://www.readbyqxmd.com/read/29228071/associations-between-tau-%C3%AE-amyloid-and-cognition-in-parkinson-disease
#6
Joseph R Winer, Anne Maass, Peter Pressman, Jordan Stiver, Daniel R Schonhaut, Suzanne L Baker, Joel Kramer, Gil D Rabinovici, William J Jagust
Importance: Multiple disease processes are associated with cognitive impairment in Parkinson disease (PD), including Lewy bodies, cerebrovascular disease, and Alzheimer disease. It remains unknown whether tau pathology relates to cognition in patients with PD without dementia. Objective: To compare tau aggregation in patients with PD who are cognitively normal (PD-CN), patients with PD with mild cognitive impairment (PD-MCI), and healthy control participants, and evaluate the relationships between β-amyloid (Aβ), tau, and cognition in patients with PD who did not have dementia...
December 11, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/29223171/caregiver-burden-sleep-quality-depression-and-anxiety-in-dementia-caregivers-a-comparison-of-frontotemporal-lobar-degeneration-dementia-with-lewy-bodies-and-alzheimer-s-disease
#7
Shuai Liu, Jing Liu, Xiao-Dan Wang, Zhihong Shi, Yuying Zhou, Jing Li, Tao Yu, Yong Ji
BACKGROUND: Very few recent studies are available that compare caregiver burden, sleep quality, and stress in caregivers of different types of dementia. We aimed to investigate caregiver burden, sleep quality, and stress in caregivers of patients with frontotemporal lobar degeneration and dementia with Lewy bodies, as compared with caregivers of patients with Alzheimer's disease. METHODS: This study was carried out from March 2011 to January 2014. In total, 492 dyads of patient and caregiver (frontotemporal lobar degeneration, n = 131; dementia with Lewy bodies, n = 36; Alzheimer's disease, n = 325) participated in this study...
December 10, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/29222591/dementia-with-lewy-bodies-and-parkinson-s-disease-dementia-current-concepts-and-controversies
#8
REVIEW
Kurt A Jellinger
Dementia with Lewy bodies (DLB) and Parkinson's disease-dementia (PDD), although sharing many clinical, neurochemical and morphological features, according to DSM-5, are two entities of major neurocognitive disorders with Lewy bodies of unknown etiology. Despite considerable clinical overlap, their diagnosis is based on an arbitrary distinction between the time of onset of motor and cognitive symptoms: dementia often preceding parkinsonism in DLB and onset of cognitive impairment after onset of motor symptoms in PDD...
December 8, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/29218503/differential-alterations-in-metabolism-and-proteolysis-related-proteins-in-human-parkinson-s-disease-substantia-nigra
#9
Edna Grünblatt, Josefine Ruder, Camelia Maria Monoranu, Peter Riederer, Moussa Bh Youdim, Silvia A Mandel
Parkinson's disease is the most common neurodegenerative disorder after Alzheimer's disease, with the majority of cases being sporadic or "idiopathic". The aetiology of the sporadic form is still unknown, but there is a broad consensus that Parkinson's disease involves multiple pathways. In previous human post-mortem studies investigating substantia nigra of parkinsonian subjects, gene expression alterations in various metabolic pathways including protein folding, trafficking, aggregation, ubiquitination and oxidative stress were found...
December 7, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/29217406/lncrna-snhg1-promotes-%C3%AE-synuclein-aggregation-and-toxicity-by-targeting-mir-15b-5p-to-activate-siah1-in-human-neuroblastoma-sh-sy5y-cells
#10
Yuan Chen, Ya-Jun Lian, Yun-Qing Ma, Chuan-Jie Wu, Ya-Ke Zheng, Nan-Chang Xie
Numerous long non-coding RNAs (lncRNAs) have been identified as aberrantly expressed in Parkinson's disease (PD). However, limited knowledge is available concerning the roles of dysregulated lncRNAs and the underlying molecular regulatory mechanism in the pathological process of PD. In this study, we found that lncRNA small nucleolar RNA host gene 1 (SNHG1) and seven in absentia homolog 1 (SIAH1) were upregulated, but microRNA-15b-5p (miR-15b-5p) was downregulated in SH-SY5Y cells pretreated with MPP+, as well as in MPTP-induced mouse model of PD...
December 4, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/29216908/clinical-and-neuropathological-features-of-als-ftd-with-tia1-mutations
#11
Veronica Hirsch-Reinshagen, Cyril Pottier, Alexandra M Nicholson, Matt Baker, Ging-Yuek R Hsiung, Charles Krieger, Pheth Sengdy, Kevin B Boylan, Dennis W Dickson, Marsel Mesulam, Sandra Weintraub, Eileen Bigio, Lorne Zinman, Julia Keith, Ekaterina Rogaeva, Sasha A Zivkovic, David Lacomis, J Paul Taylor, Rosa Rademakers, Ian R A Mackenzie
Mutations in the stress granule protein T-cell restricted intracellular antigen 1 (TIA1) were recently shown to cause amyotrophic lateral sclerosis (ALS) with or without frontotemporal dementia (FTD). Here, we provide detailed clinical and neuropathological descriptions of nine cases with TIA1 mutations, together with comparisons to sporadic ALS (sALS) and ALS due to repeat expansions in C9orf72 (C9orf72+). All nine patients with confirmed mutations in TIA1 were female. The clinical phenotype was heterogeneous with a range in the age at onset from late twenties to the eighth decade (mean = 60 years) and disease duration from one to 6 years (mean = 3 years)...
December 7, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29213511/lewy-and-his-inclusion-bodies-discovery-and-rejection
#12
Eliasz Engelhardt, Marleide da Mota Gomes
Fritz Jacob Heinrich Lewy described the pathology of Paralysis agitans [Parkinson disease] and was the first to identify eosinophilic inclusion bodies in neurons of certain brain nuclei, later known as Lewy bodies, the pathological signature of the Lewy body diseases. In 1912, he published his seminal study, followed soon after by an update paper, and 10 years later, in 1923, by his voluminous book, where he exhaustively described the subject. The publication provided extensive information on the pathology of Paralysis agitans, and the entirely novel finding of eosinophilic inclusion bodies, which would become widely recognized and debated in the future...
April 2017: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29198173/autophagy-inhibition-promotes-snca-alpha-synuclein-release-and-transfer-via-extracellular-vesicles-with-a-hybrid-autophagosome-exosome-like-phenotype
#13
Georgia Minakaki, Stefanie Menges, Agnes Kittel, Evangelia Emmanouilidou, Iris Schaeffner, Katalin Barkovits, Anna Bergmann, Edward Rockenstein, Anthony Adame, Franz Marxreiter, Brit Mollenhauer, Douglas Galasko, Edit Irén Buzás, Ursula Schlötzer-Schrehardt, Katrin Marcus, Wei Xiang, Dieter Chichung Lie, Kostas Vekrellis, Eliezer Masliah, Jürgen Winkler, Jochen Klucken
The autophagy-lysosome pathway (ALP) regulates intracellular homeostasis of the cytosolic protein SNCA/alpha-synuclein and is impaired in synucleinopathies, including Parkinson disease and dementia with Lewy bodies (DLB). Emerging evidence suggests that ALP influences SNCA release, but the underlying cellular mechanisms are not well understood. Several studies identified SNCA in exosome/extracellular vesicle (EV) fractions. EVs are generated in the multivesicular body compartment and either released upon its fusion with the plasma membrane, or cleared via the ALP...
December 4, 2017: Autophagy
https://www.readbyqxmd.com/read/29196808/propagation-of-alpha-synuclein-pathology-from-the-olfactory-bulb-possible-role-in-the-pathogenesis-of-dementia-with-lewy-bodies
#14
REVIEW
María Graciela Cersosimo
Olfactory limbic structures, like the amygdala, the entorhinal, and the piriform cortices, are closely involved in cognitive processes. Thus, besides olfactory dysfunctions, it is conceivable that the compromise of these structures can lead to cognitive impairment. The olfactory bulb is affected by alpha-synuclein pathology in almost all cases of both Parkinson's disease and dementia with Lewy bodies. The clinical distinction between these disorders relies on the timing in the appearance of dementia in relationship to motor symptoms...
December 1, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/29196214/the-lysosomal-enzyme-alpha-galactosidase-a-is-deficient-in-parkinson-s-disease-brain-in-association-with-the-pathologic-accumulation-of-alpha-synuclein
#15
Michael P Nelson, Michel Boutin, Tonia E Tse, Hailin Lu, Emily D Haley, Xiaosen Ouyang, Jianhua Zhang, Christiane Auray-Blais, John J Shacka
The aberrant accumulation of alpha-synuclein (α-syn) is believed to contribute to the onset and pathogenesis of Parkinson's disease (PD). The autophagy-lysosome pathway (ALP) is responsible for the high capacity clearance of α-syn. ALP dysfunction is documented in PD and pre-clinical evidence suggests that inhibiting the ALP promotes the pathological accumulation of α-syn. We previously identified the pathological accumulation of α-syn in the brains of mice deficient for the soluble lysosomal enzyme alpha-Galactosidase A (α-Gal A), a member of the glycosphingolipid metabolism pathway...
December 2, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29195744/discrimination-of-atypical-parkinsonisms-with-transcranial-magnetic-stimulation
#16
Alberto Benussi, Valentina Dell'Era, Valentina Cantoni, Clarissa Ferrari, Salvatore Caratozzolo, Luca Rozzini, Antonella Alberici, Alessandro Padovani, Barbara Borroni
BACKGROUND: Differential diagnosis of atypical parkinsonian disorders, i.e. dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) still remains problematic. Furthermore, DLB may overlap with Alzheimer's disease (AD) in the early stages of disease. OBJECTIVE: To determine whether transcranial magnetic stimulation (TMS) can be used to classify atypical parkinsonian disorders and AD. METHODS: A paired-pulse TMS multi-paradigm approach assessing multiple intracortical circuits, as short interval intracortical inhibition-facilitation and short latency afferent inhibition, was used to model a decision tree analysis and determine diagnostic accuracy in classifying different neurodegenerative disorders...
November 22, 2017: Brain Stimulation
https://www.readbyqxmd.com/read/29193464/functional-connectivity-in-dementia-with-lewy-bodies-a-within-and-between-network-analysis
#17
Julia Schumacher, Luis R Peraza, Michael Firbank, Alan J Thomas, Marcus Kaiser, Peter Gallagher, John T O'Brien, Andrew M Blamire, John-Paul Taylor
Dementia with Lewy bodies (DLB) is a common form of dementia and is characterized by cognitive fluctuations, visual hallucinations, and Parkinsonism. The phenotypic expression of the disease may, in part, relate to alterations in functional connectivity within and between brain networks. This resting-state study sought to clarify this in DLB, how networks differed from Alzheimer's disease (AD), and whether they were related to clinical symptoms in DLB. Resting-state networks were estimated using independent component analysis...
November 29, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/29189301/assessing-fitness-to-drive-in-patients-with-different-types-of-dementia
#18
Dafne Piersma, Anselm B M Fuermaier, Dick De Waard, Ragnhild J Davidse, Jolieke De Groot, Michelle J A Doumen, Ruud A Bredewoud, René Claesen, Afina W Lemstra, Philip Scheltens, Annemiek Vermeeren, Rudolf Ponds, Frans Verhey, Peter P De Deyn, Wiebo H Brouwer, Oliver Tucha
Dementia is a risk factor for unsafe driving. Therefore, an assessment strategy has recently been developed for the prediction of fitness to drive in patients with the Alzheimer disease (AD). The aim of this study was to investigate whether this strategy is also predictive of fitness to drive in patients with non-AD dementia, that is, vascular dementia, frontotemporal dementia, and dementia with Lewy bodies. Predictors were derived from 3 types of assessment: clinical interviews, neuropsychological tests, and driving simulator rides...
November 17, 2017: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29189256/enteric-pathologic-manifestations-of-alpha-synucleinopathies
#19
Michael Punsoni, Joseph H Friedman, Murray Resnick, John E Donahue, Dong Fang Yang, Edward G Stopa
BACKGROUND: Gastrointestinal (GI) symptoms are common in Parkinson disease (PD), often preceding neurological manifestations; however, early diagnostic utility of GI biopsies remains controversial. Studies suggest aberrant deposition of alpha-synuclein (α-syn) follows step-wise progression in central nervous system though histologic interpretation of normal and aberrant staining patterns have shown variable results. This study examines whether GI α-syn mRNA expression combined with standard α-syn immunohistochemical staining enhance the role of GI biopsy in PD...
November 20, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29187334/cognitive-profile-in-prodromal-disease-dementia-with-lewy-bodies
#20
Jennifer Kemp, Nathalie Philippi, Clélie Phillipps, Anne Botzung, Frédéric Blanc
This paper reviews findings on cognition in prodromal dementia with Lewy bodies (DLB). The issue of differential diagnosis between DLB and Alzheimer's disease (AD) on the one hand, and Parkinson's disease (PD) on the other hand, at prodromal stages of the diseases is discussed. The cognitive profile of prodromal DLB is also evaluated in terms of cognitive deficits and « cognitive weaknesses » (low performances although in normal range). Findings suggest that visuo-constructive tests and verbal fluency are particularly relevant to dfferentiate DLB from AD...
December 1, 2017: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
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