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Lewy body dementia

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https://www.readbyqxmd.com/read/28324300/%C3%AE-synuclein-and-parkinsonism-updates-and-future-perspectives
#1
REVIEW
Kaie Rosborough, Neha Patel, Lorraine V Kalia
Mutations in the SNCA gene, which encodes the α-synuclein protein, were the first discovered genetic causes of familial parkinsonism with Lewy pathology. To date, six different SNCA missense mutations as well as multiplications are known to cause parkinsonism. For this review, we performed a literature search to identify all published cases of SNCA-related parkinsonism to provide an updated summary of the clinical and neuropathological features of parkinsonism due to SNCA mutations. Familial parkinsonism associated with SNCA is rare, but α-synuclein aggregation is a core feature of sporadic parkinsonism, including Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy...
April 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28323683/genetic-alzheimer-disease-and-sporadic-dementia-with-lewy-bodies-a-comorbidity-presenting-as-primary-progressive-aphasia
#2
Tereza Picková, Radoslav Matěj, Ondrej Bezdicek, Jiří Keller, Julie van der Zee, Christine Van Broeckhoven, Zsolt Cséfalvay, Robert Rusina
We report a 44-year-old woman, with a family history of early-onset dementia, presenting with primary progressive aphasia. This clinically variable syndrome has multiple underlying pathologies, and correlations between clinical manifestations and postmortem neuropathologic findings are controversial. Our patient suffered worsening language impairment with major word-finding difficulties but preserved comprehension. She also developed episodic memory impairment. Her condition progressed to dementia with behavioral changes...
March 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28321951/transcriptional-network-analysis-in-frontal-cortex-in-lewy-body-diseases-with-focus-on-dementia-with-lewy-bodies
#3
Gabriel Santpere, Paula Garcia-Esparcia, Pol Andres-Benito, Belen Lorente-Galdos, Arcadi Navarro, Isidro Ferrer
The present study investigates global transcriptional changes in frontal cortex area 8 in incidental Lewy Body disease (iLBD), Parkinson disease (PD) and Dementia with Lewy bodies (DLB). We identified different co-expressed gene sets associated with disease stages, and gene ontology categories enriched in gene modules and differentially expressed genes including modules or gene clusters correlated to iLBD comprising upregulated dynein genes and taste receptors, and down-regulated innate inflammation. Focusing on DLB, we found modules with genes significantly enriched in functions related to RNA and DNA production, mitochondria and energy metabolism, purine metabolism, chaperone and protein folding system, and synapses and neurotransmission (particularly the GABAergic system)...
March 21, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28317651/clinical-validity-of-presynaptic-dopaminergic-imaging-with%C3%A2-123-i-ioflupane%C3%A2-and-noradrenergic-imaging-with-123-i-mibg-in-the-differential-diagnosis-between-alzheimer-s-disease-and-dementia-with-lewy-bodies-in-the-context-of-a-structured-5-phase-development
#4
REVIEW
Ida Sonni, Osman Ratib, Marina Boccardi, Agnese Picco, Karl Herholz, Flavio Nobili, Andrea Varrone
The use of biomarkers (BMs) for accurate diagnosis of Alzheimer's disease (AD) has been proposed by recent diagnostic criteria; however, their maturity is not sufficient to grant implementation in the clinical routine. A proper diagnostic process requires not only confirmation of the disease but also the exclusion of similar disorders entering differential diagnosis, like dementia with Lewy bodies (DLB). This review is aimed at evaluating the clinical validity of (123)I-ioflupane brain single photon emission tomography and (123)I-MIBG cardiac scintigraphy as imaging BMs for DLB...
April 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28315409/in-vivo-tau-pet-imaging-in-dementia-pathophysiology-radiotracer-quantification-and-a-systematic-review-of-clinical-findings
#5
REVIEW
Benjamin Hall, Elijah Mak, Simon Cervenka, Franklin I Aigbirhio, James B Rowe, John T O'Brien
In addition to the deposition of β-amyloid plaques, neurofibrillary tangles composed of aggregated hyperphosphorylated tau are one of the pathological hallmarks of Alzheimer's disease and other neurodegenerative disorders. Until now, our understanding about the natural history and topography of tau deposition has only been based on post-mortem and cerebrospinal fluid studies, and evidence continues to implicate tau as a central driver of downstream neurodegenerative processes and cognitive decline. Recently, it has become possible to assess the regional distribution and severity of tau burden in vivo with the development of novel radiotracers for positron emission tomography (PET) imaging...
March 14, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28304294/long-term-cognitive-decline-in-dementia-with-lewy-bodies-in-a-large-multicenter-international-cohort
#6
Milica G Kramberger, Bjørn Auestad, Sara Garcia-Ptacek, Carla Abdelnour, Josep Garre Olmo, Zuzana Walker, Afina W Lemstra, Elisabet Londos, Frederic Blanc, Laura Bonanni, Ian McKeith, Bengt Winblad, Frank Jan de Jong, Flavio Nobili, Elka Stefanova, Maria Petrova, Cristian Falup-Pecurariu, Irena Rektorova, Sevasti Bostantjopoulou, Roberta Biundo, Daniel Weintraub, Dag Aarsland
BACKGROUND/OBJECTIVE: The aim of this study was to describe the rate and clinical predictors of cognitive decline in dementia with Lewy bodies (DLB), and compare the findings with Alzheimer's disease (AD) and Parkinson's disease dementia (PDD) patients. METHODS: Longitudinal scores for the Mini-Mental State Examination (MMSE) in 1,290 patients (835 DLB, 198 PDD, and 257 AD) were available from 18 centers with up to three years longitudinal data. Linear mixed effects analyses with appropriate covariates were used to model MMSE decline over time...
March 10, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28302161/cognitive-profile-in-prodromal-dementia-with-lewy-bodies
#7
Jennifer Kemp, Nathalie Philippi, Clélie Phillipps, Catherine Demuynck, Timothée Albasser, Catherine Martin-Hunyadi, Catherine Schmidt-Mutter, Benjamin Cretin, Frédéric Blanc
BACKGROUND: Cortical and subcortical cognitive impairments have been found in dementia with Lewy bodies (DLB). Roughly, they comprise visuoconstructive and executive dysfunction, whereas memory would remain relatively spared. However, the cognitive profile of patients with prodromal DLB remains poorly illustrated to date. METHODS: We included 37 patients with prodromal DLB (age 67.2 ± 8.6 years, 18 men, Mini Mental State Examination [MMSE] score 27.4 ± 2) and 29 healthy control subjects (HCs; age 68...
March 16, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28295599/gender-difference-in-the-association-and-presentation-of-visual-hallucinations-in-dementia-with-lewy-bodies-a-cross-sectional-study
#8
Pai-Yi Chiu, Po-Ren Teng, Cheng-Yu Wei, Chein-Wei Wang, Chun-Tang Tsai
OBJECTIVE: Visual hallucinations (VHs) are among the most striking features of dementia with Lewy bodies (DLB). We investigated gender differences in the association and presentation of VHs in DLB. METHODS: A cross-sectional analysis of baseline data from a prospective, longitudinal study on dementia was performed. Cumulative frequency, 1-month frequency, and phenomenology of VHs were summarized and compared between female and male patients with DLB. Gender differences in the factors associated with VHs were investigated in patients with and without hallucinations...
March 13, 2017: International Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28285306/extremely-low-prevalence-of-amyloid-positron-emission-tomography-positivity-in-parkinson-s-disease-without-dementia
#9
Kyoko Mashima, Daisuke Ito, Masashi Kameyama, Takashi Osada, Hajime Tabuchi, Yoshihiro Nihei, Takahito Yoshizaki, Eri Noguchi, Mariko Tanikawa, Takashi Iizuka, Yugaku Date, Yuji Ogata, Tadaki Nakahara, Yu Iwabuchi, Masahiro Jinzaki, Koji Murakami, Norihiro Suzuki
BACKGROUND: Most cases of dementia with Lewy bodies (DLB) show Alzheimer's disease pathology-like senile plaques and neurofibrillary tangles. Several studies have also revealed a high prevalence of positive amyloid imaging with positron emission tomography (PET) in DLB and moderate prevalence in Parkinson's disease (PD) with dementia. However, it remains unclear in PD without dementia as to when the brain β amyloid (Aβ) burden begins and progresses. Our study aimed to determine the prevalence of Aβ deposition in PD without dementia using amyloid PET...
March 11, 2017: European Neurology
https://www.readbyqxmd.com/read/28283027/integrated-analysis-of-genetic-behavioral-and-biochemical-data-implicates-neural-stem-cell-induced-changes-in-immunity-neurotransmission-and-mitochondrial-function-in-dementia-with-lewy-body-mice
#10
Anita Lakatos, Natalie R S Goldberg, Mathew Blurton-Jones
We previously demonstrated that transplantation of murine neural stem cells (NSCs) can improve motor and cognitive function in a transgenic model of Dementia with Lewy Bodies (DLB). These benefits occurred without changes in human α-synuclein pathology and were mediated in part by stem cell-induced elevation of brain-derived neurotrophic factor (BDNF). However, instrastriatal NSC transplantation likely alters the brain microenvironment via multiple mechanisms that may synergize to promote cognitive and motor recovery...
March 10, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28282814/the-synucleinopathies-twenty-years-on
#11
Michel Goedert, Ross Jakes, Maria Grazia Spillantini
In 2017, it is two hundred years since James Parkinson provided the first complete clinical description of the disease named after him, fifty years since the introduction of high-dose D,L-DOPA treatment and twenty years since α-synuclein aggregation came to the fore. In 1998, multiple system atrophy joined Parkinson's disease and dementia with Lewy bodies as the third major synucleinopathy. Here we review our work, which led to the identification of α-synuclein in Lewy bodies, Lewy neurites and Papp-Lantos bodies, as well as what has happened since...
2017: Journal of Parkinson's Disease
https://www.readbyqxmd.com/read/28272788/corrigendum-caregiver-burden-in-family-carers-of-people-with-dementia-with-lewy-bodies-and-alzheimer-s-disease
#12
(no author information available yet)
No abstract text is available yet for this article.
April 2017: International Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28269775/reduction-of-rpt6-s8-a-proteasome-component-and-proteasome-activity-in-the-cortex-is-associated-with-cognitive-impairment-in-lewy-body-dementia
#13
Amani Alghamdi, Julie Vallortigara, David R Howlett, Martin Broadstock, Tibor Hortobágyi, Clive Ballard, Alan J Thomas, John T O'Brien, Dag Aarsland, Johannes Attems, Paul T Francis, David R Whitfield
Lewy body dementia is the second most common neurodegenerative dementia and is pathologically characterized by α-synuclein positive cytoplasmic inclusions, with varying amounts of amyloid-β (Aβ) and hyperphosphorylated tau (tau) aggregates in addition to synaptic loss. A dysfunctional ubiquitin proteasome system (UPS), the major proteolytic pathway responsible for the clearance of short lived proteins, may be a mediating factor of disease progression and of the development of α-synuclein aggregates. In the present study, protein expression of a key component of the UPS, the RPT6 subunit of the 19S regulatory complex was determined...
March 1, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28265813/quantitative-neuropathology-an-update-on-automated-methodologies-and-implications-for-large-scale-cohorts
#14
Lauren Walker, Kirsty E McAleese, Mary Johnson, Ahmad A Khundakar, Daniel Erskine, Alan J Thomas, Ian G McKeith, Johannes Attems
A tissue microarray (TMA) has previously been developed for use in assessment of neurodegenerative diseases. We investigated the variation of pathology loads in semi-quantitative score categories and how pathology load related to disease progression. Post-mortem tissue from 146 cases were used; Alzheimer's disease (AD) (n = 36), Lewy body disease (LBD) (n = 56), mixed AD/dementia with Lewy bodies (n = 14) and controls (n = 40). TMA blocks (one per case) were constructed using tissue cores from 15 brain regions including cortical and subcortical regions...
March 6, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28259856/7t-mri-for-neurodegenerative-dementias-in-vivo-a-systematic-review-of-the-literature
#15
REVIEW
Elizabeth Frances McKiernan, John Tiernan O'Brien
The spatial resolution of 7T MRI approaches the scale of pathologies of interest in degenerative brain diseases, such as amyloid plaques and changes in cortical layers and subcortical nuclei. It may reveal new information about neurodegenerative dementias, although challenges may include increased artefact production and more adverse effects. We performed a systematic review of papers investigating Alzheimer's disease (AD), Lewy body dementia (LBD), frontotemporal dementia (FTD) and Huntington's disease (HD) in vivo using 7T MRI...
March 4, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28255741/is-pure-autonomic-failure-an-early-marker-for-parkinson-disease-dementia-with-lewy-bodies-and-multiple-system-atrophy-and-other-updates-on-recent-autonomic-research
#16
EDITORIAL
Srikanth Muppidi, Mitchell G Miglis
No abstract text is available yet for this article.
March 2, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/28253276/clinical-and-neuroimaging-characteristics-of-chinese-dementia-with-lewy-bodies
#17
Shuai Liu, Xiao-Dan Wang, Ying Wang, Zhihong Shi, Li Cai, Shuling Liu, Tong Han, Yuying Zhou, Xinping Wang, Shuo Gao, Yong Ji
Dementia with Lewy bodies (DLB) is the second most common subtype of degenerative dementia. To our knowledge, available information about the clinical features of DLB in China remains limited. Our study therefore aimed to address this issue. Thirty-seven Chinese patients with probable DLB were recruited for this study. All subjects underwent neuropsychological assessment by trained neurologists, as well as undergoing MRI, 11C-PIB PET scans for Aβ deposition and 18F-FDG PET scans for regional cerebral glucose metabolism...
2017: PloS One
https://www.readbyqxmd.com/read/28244178/evaluation-of-an-optimized-18-f-fluoro-deoxy-glucose-positron-emission-tomography-voxel-wise-method-to-early-support-differential-diagnosis-in-atypical-parkinsonian-disorders
#18
S P Caminiti, P Alongi, L Majno, M A Volontè, C Cerami, L Gianolli, G Comi, D Perani
BACKGROUND AND PURPOSE: Atypical Parkinsonian disorders (APD) frequently overlap in clinical presentations, making the differential diagnosis challenging in the early stages. The present study aimed to evaluate the accuracy of the [(18) F]fluoro-deoxy-glucose positron emission tomography Statistical Parametric Mapping (SPM) optimized procedure in supporting the early and differential diagnosis of APD. METHODS: Seventy patients with possible APD were retrospectively included from a large clinical cohort...
February 28, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28243755/differential-diagnosis-between-patients-with-probable-alzheimer-s-disease-parkinson-s-disease-dementia-or-dementia-with-lewy-bodies-and-frontotemporal-dementia-behavioral-variant-using-quantitative-electroencephalographic-features
#19
Heinrich Garn, Carmina Coronel, Markus Waser, Georg Caravias, Gerhard Ransmayr
The objective of this work was to develop and evaluate a classifier for differentiating probable Alzheimer's disease (AD) from Parkinson's disease dementia (PDD) or dementia with Lewy bodies (DLB) and from frontotemporal dementia, behavioral variant (bvFTD) based on quantitative electroencephalography (QEEG). We compared 25 QEEG features in 61 dementia patients (20 patients with probable AD, 20 patients with PDD or probable DLB (DLBPD), and 21 patients with bvFTD). Support vector machine classifiers were trained to distinguish among the three groups...
February 27, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28243754/differentiation-of-atypical-parkinson-syndromes
#20
REVIEW
Günter U Höglinger, Jan Kassubek, Ilona Csoti, Reinhard Ehret, Heinz Herbst, Ingmar Wellach, Jürgen Winkler, Wolfgang H Jost
In distinction to idiopathic Parkinson's disease (PD), the diagnosis of atypical Parkinson syndromes comprises dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). We set out to write a state-of-the-art guideline as to which investigations and examinations help to differentiate PD vs. atypical Parkinson syndromes in clinical routine.
February 27, 2017: Journal of Neural Transmission
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