keyword
https://read.qxmd.com/read/38499535/htra1-disaggregates-%C3%AE-synuclein-amyloid-fibrils-and-converts-them-into-non-toxic-and-seeding-incompetent-species
#21
JOURNAL ARTICLE
Sheng Chen, Anuradhika Puri, Braxton Bell, Joseph Fritsche, Hector H Palacios, Maurie Balch, Macy L Sprunger, Matthew K Howard, Jeremy J Ryan, Jessica N Haines, Gary J Patti, Albert A Davis, Meredith E Jackrel
Parkinson's disease (PD) is closely linked to α-synuclein (α-syn) misfolding and accumulation in Lewy bodies. The PDZ serine protease HTRA1 degrades fibrillar tau, which is associated with Alzheimer's disease, and inactivating mutations to mitochondrial HTRA2 are implicated in PD. Here, we report that HTRA1 inhibits aggregation of α-syn as well as FUS and TDP-43, which are implicated in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. The protease domain of HTRA1 is necessary and sufficient for inhibiting aggregation, yet this activity is proteolytically-independent...
March 18, 2024: Nature Communications
https://read.qxmd.com/read/38499333/we-need-to-talk-about-brain-donation
#22
EDITORIAL
Martin R Turner
No abstract text is available yet for this article.
March 18, 2024: Practical Neurology
https://read.qxmd.com/read/38497928/sex-specific-neuropsychological-correlates-of-apathy-and-depression-across-neurodegenerative-disorders
#23
JOURNAL ARTICLE
Daniel Kapustin, Shankar Tumati, Melissa Wong, Nathan Herrmann, Roger A Dixon, Dallas Seitz, Mark J Rapoport, Krista L Lanctôt
BACKGROUND: Apathy and depression are common neuropsychiatric symptoms across neurodegenerative disorders and are associated with impairment in several cognitive domains, yet little is known about the influence of sex on these relationships. OBJECTIVES: We examined the relationship between these symptoms with neuropsychological performance across a combined cohort with mild or major neurodegenerative disorders, then evaluated the impact of sex. DESIGN, SETTING AND PARTICIPANTS: We conducted a cohort analysis of participants in the COMPASS-ND study with mild cognitive impairment (MCI), vascular MCI, Alzheimer's disease, mixed dementia, Parkinson's disease, frontotemporal dementia, and cognitively unimpaired (CU) controls...
March 2024: International Journal of Geriatric Psychiatry
https://read.qxmd.com/read/38497896/eeg-rhythmic-and-arrhythmic-spectral-components-and-functional-connectivity-at-resting-state-may-predict-the-development-of-synucleinopathies-in-idiopathic-rem-sleep-behavior-disorder
#24
JOURNAL ARTICLE
J Hernandez, J-M Lina, J Dubé, A Lafrenière, J-F Gagnon, J-Y Montplaisir, R B Postuma, J Carrier
STUDY OBJECTIVES: Idiopathic/isolated REM-sleep behavior disorder (iRBD) often precedes the onset of synucleinopathies. Here, we investigated whether baseline resting-state EEG advanced spectral power and functional connectivity differ between iRBD patients who converted towards a synucleinopathy at follow-up and those who did not. METHODS: Eighty-one participants with iRBD (66.89±6.91 years) underwent a baseline resting-state EEG recording, a neuropsychological assessment and a neurological examination...
March 18, 2024: Sleep
https://read.qxmd.com/read/38497239/place-of-death-in-parkinson-s-disease-and-related-disorders-in-england-and-wales-post-pandemic-trends-and-implications-for-care-planning
#25
JOURNAL ARTICLE
Elisabeth Wilson, Amy Baker, Lauren Stockley, Victoria Allgar, Edward Richfield
BACKGROUND: With growing emphasis on palliative care for neurodegenerative conditions, understanding trends in place of death helps improve quality of end-of-life care for people with Parkinson's disease and related disorders (PDRDs), focusing allocation of resources and training and identifying inequalities. OBJECTIVES: Review national and regional place of death trends for people with PDRD including pre- and post-pandemic trends. METHODS: Mortality data for England and Wales (March 2018 and July 2022) were analysed with summary statistics and interrupted time series, exploring place of death for those who died with PDRD, with and without coexisting dementia, with reference to all deaths in England and Wales...
March 1, 2024: Age and Ageing
https://read.qxmd.com/read/38497236/delirium-is-more-common-and-associated-with-worse-outcomes-in-parkinson-s-disease-compared-to-older-adult-controls-results-of-two-prospective-longitudinal-cohort-studies
#26
JOURNAL ARTICLE
Florence Gerakios, Alison J Yarnall, Gemma Bate, Laura Wright, Daniel Davis, Blossom C M Stephan, Louise Robinson, Carol Brayne, Glenn Stebbins, John-Paul Taylor, David J Burn, Louise M Allan, Sarah J Richardson, Rachael A Lawson
BACKGROUND: Inpatient prevalence of Parkinson's disease (PD) delirium varies widely across the literature. Delirium in general older populations is associated with adverse outcomes, such as increased mortality, dementia, and institutionalisation. However, to date there are no comprehensive prospective studies in PD delirium. This study aimed to determine delirium prevalence in hospitalised PD participants and the association with adverse outcomes, compared to a control group of older adults without PD...
March 1, 2024: Age and Ageing
https://read.qxmd.com/read/38496612/alpha-synuclein-regulates-the-repair-of-genomic-dna-double-strand-breaks-in-a-dna-pk-cs-dependent-manner
#27
Elizabeth P Rose, Valerie R Osterberg, Jovin S Banga, Vera Gorbunova, Vivek K Unni
α-synuclein (αSyn) is a presynaptic and nuclear protein that aggregates in important neurodegenerative diseases such as Parkinson's Disease (PD), Parkinson's Disease Dementia (PDD) and Lewy Body Dementia (LBD). Our past work suggests that nuclear αSyn may regulate forms of DNA double-strand break (DSB) repair in HAP1 cells after DNA damage induction with the chemotherapeutic agent bleomycin 1 . Here, we report that genetic deletion of αSyn specifically impairs the non-homologous end-joining (NHEJ) pathway of DSB repair using an extrachromosomal plasmid-based repair assay in HAP1 cells...
March 4, 2024: bioRxiv
https://read.qxmd.com/read/38496508/deep-sequencing-of-proteotoxicity-modifier-genes-uncovers-a-presenilin-2-beta-amyloid-actin-genetic-risk-module-shared-among-alpha-synucleinopathies
#28
Sumaiya Nazeen, Xinyuan Wang, Dina Zielinski, Isabel Lam, Erinc Hallacli, Ping Xu, Elizabeth Ethier, Ronya Strom, Camila A Zanella, Vanitha Nithianandam, Dylan Ritter, Alexander Henderson, Nathalie Saurat, Jalwa Afroz, Andrew Nutter-Upham, Hadar Benyamini, Joseph Copty, Shyamsundar Ravishankar, Autumn Morrow, Jonathan Mitchel, Drew Neavin, Renuka Gupta, Nona Farbehi, Jennifer Grundman, Richard H Myers, Clemens R Scherzer, John Q Trojanowski, Vivianna M Van Deerlin, Antony A Cooper, Edward B Lee, Yaniv Erlich, Susan Lindquist, Jian Peng, Daniel H Geschwind, Joseph Powell, Lorenz Studer, Mel B Feany, Shamil R Sunyaev, Vikram Khurana
Whether neurodegenerative diseases linked to misfolding of the same protein share genetic risk drivers or whether different protein-aggregation pathologies in neurodegeneration are mechanistically related remains uncertain. Conventional genetic analyses are underpowered to address these questions. Through careful selection of patients based on protein aggregation phenotype (rather than clinical diagnosis) we can increase statistical power to detect associated variants in a targeted set of genes that modify proteotoxicities...
March 7, 2024: bioRxiv
https://read.qxmd.com/read/38495226/adult-onset-leukodystrophy-due-to-tmem63a-variant-presenting-with-rapidly-progressive-dementia-with-parkinsonism
#29
JOURNAL ARTICLE
Divyani Garg, Ayush Agarwal, Ajay Garg, Achal Kumar Srivastava
No abstract text is available yet for this article.
2024: Annals of Indian Academy of Neurology
https://read.qxmd.com/read/38494747/rapid-eye-movement-sleep-behavior-disorder-and-its-relation-to-parkinson-s-disease-the-potential-of-graph-measures-as-brain-biomarkers-to-identify-the-underlying-physiopathology-of-the-disorder
#30
REVIEW
Milad Najafzadeh, Fatemeh Mohammadian, Sara Mirabian, Zohre Ganji, Hossein Akbari, Masoud Rezaie, Esmaeil Ranjbar, Hoda Zare, Shahrokh Nasseri, Luigi Ferini-Strambi
Rapid eye movement behavior disorder (RBD) is a parasomnia characterized by the loss of skeletal muscle atonia during the rapid eye movement (REM) sleep phase. On the other hand, idiopathic RDB (iRBD) is considered the prelude of the various α-synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies and multiple system atrophy. Consequently, over 40% of patients eventually develop PD. Recent neuroimaging studies utilizing structural magnetic resonance imaging (s-MRI), diffusion-weighted imaging (DWI), and functional magnetic resonance imaging (fMRI) with graph theoretical analysis have demonstrated that patients with iRBD and Parkinson's disease have extensive brain abnormalities...
March 2024: Brain and Behavior
https://read.qxmd.com/read/38494398/correlation-between-clinical-and-neuropathological-subtypes-of-progressive-supranuclear-palsy
#31
JOURNAL ARTICLE
Ryuichi Koizumi, Akagi Akio, Yuichi Riku, Hiroaki Miyahara, Jun Sone, Fumiaki Tanaka, Mari Yoshida, Yasushi Iwasaki
INTRODUCTION: Progressive supranuclear palsy (PSP) is characterized by pathology prominently in the basal ganglia, the tegmentum of the brainstem, and the frontal cortex. However, pathology varies according to clinical features. This study aimed to statistically verify the correspondence between the clinical and pathological subtypes of PSP. METHODS: We identified patients with a pathological diagnosis of PSP and classified the eight clinical subtypes of the Movement Disorders Society criteria for the clinical diagnosis of PSP (MDS-PSP criteria) into the Richardson, Akinesia, and Cognitive groups...
March 9, 2024: Parkinsonism & related Disorders
https://read.qxmd.com/read/38492784/are-age-related-neurodegenerative-diseases-caused-by-a-lack-of-the-diet-derived-compound-ergothioneine
#32
REVIEW
Barry Halliwell, Irwin Cheah
We propose that the diet-derived compound ergothioneine (ET) is an important nutrient in the human body, especially for maintenance of normal brain function, and that low body ET levels predispose humans to significantly increased risks of neurodegenerative (cognitive impairment, dementia, Parkinson's disease) and possibly other age-related diseases (including frailty, cardiovascular disease, and eye disease). Hence, restoring ET levels in the body could assist in mitigating these risks, which are rapidly increasing due to ageing populations globally...
March 14, 2024: Free Radical Biology & Medicine
https://read.qxmd.com/read/38491809/neuropsychiatric-symptoms-and-brain-morphology-in-patients-with-mild-cognitive-impairment-cerebrovascular-disease-and-parkinson-disease-a-cross-sectional-and-longitudinal-study
#33
JOURNAL ARTICLE
Neda Rashidi-Ranjbar, Nathan W Churchill, Sandra E Black, Sanjeev Kumar, Maria C Tartaglia, Morris Freedman, Anthony Lang, Thomas D L Steeves, Richard H Swartz, Gustavo Saposnik, Dametrios Sahlas, Paula McLaughlin, Sean Symons, Stephen Strother, Bruce G Pollock, Tarek K Rajji, Miracle Ozzoude, Brian Tan, Stephen R Arnott, Robert Bartha, Michael Borrie, Mario Masellis, Stephen H Pasternak, Andrew Frank, Dallas Seitz, Zahinoor Ismail, David F Tang-Wai, Leanne K Casaubon, Jennifer Mandzia, Mandar Jog, Christopher J M Scott, Dar Dowlatshahi, Ayman Hassan, David Grimes, Connie Marras, Mojdeh Zamyadi, David G Munoz, Joel Ramirez, Courtney Berezuk, Melissa Holmes, Corinne E Fischer, Tom A Schweizer
OBJECTIVES: Neuropsychiatric symptoms (NPS) increase risk of developing dementia and are linked to various neurodegenerative conditions, including mild cognitive impairment (MCI due to Alzheimer's disease [AD]), cerebrovascular disease (CVD), and Parkinson's disease (PD). We explored the structural neural correlates of NPS cross-sectionally and longitudinally across various neurodegenerative diagnoses. METHODS: The study included individuals with MCI due to AD, (n = 74), CVD (n = 143), and PD (n = 137) at baseline, and at 2-years follow-up (MCI due to AD, n = 37, CVD n = 103, and PD n = 84)...
March 2024: International Journal of Geriatric Psychiatry
https://read.qxmd.com/read/38489578/free-water-imaging-of-the-nucleus-basalis-of-meynert-in-patients-with-idiopathic-rem-sleep-behavior-disorder-and-parkinson-disease
#34
JOURNAL ARTICLE
Dongling Zhang, Liche Zhou, Chenxi Lu, Tao Feng, Jun Liu, Tao Wu
BACKGROUND AND OBJECTIVES: Cognitive impairments are common in idiopathic REM sleep behavior disorder (iRBD), in which the cholinergic degeneration of nucleus basalis of Meynert (NBM) may play an important role. However, the progressive changes of NBM, the relationship between progressive NBM degeneration and progression of cognitive impairments, and whether degeneration of the NBM can predict cognitive decline in patients with iRBD remain unclear. This study aimed to investigate the cross-sectional and longitudinal microstructural alterations in the NBM of patients with iRBD using free-water imaging and whether free water in the NBM is related to cognitive, mood, and autonomic function...
April 9, 2024: Neurology
https://read.qxmd.com/read/38488199/socioeconomic-disparities-in-neurodegenerative-disease-mortality-a-population-based-study-among-belgian-men-and-women-aged-65-or-older
#35
JOURNAL ARTICLE
Janna Dinneweth, Sylvie Gadeyne
This study investigates the association between socioeconomic position (SEP) - in terms of income and education - and mortality from neurodegenerative diseases, that is, dementia, parkinsonism, and motor neuron diseases (MNDs). We calculated age-standardized mortality rates and mortality rate ratios using log linear Poisson regression for different SEP groups, stratified by gender, age-group, and care home residency, utilizing the 2011 Belgian census linked to register data on cause-specific mortality for 2011 to 2016...
2024: Inquiry: a Journal of Medical Care Organization, Provision and Financing
https://read.qxmd.com/read/38487578/association-between-cns-active-drugs-and-risk-of-alzheimer-s-and-age-related-neurodegenerative-diseases
#36
JOURNAL ARTICLE
Helena Cortes-Flores, Georgina Torrandell-Haro, Roberta Diaz Brinton
OBJECTIVE: As neuropsychiatric conditions can increase the risk of age-related neurodegenerative diseases (NDDs), the impact of CNS-active drugs on the risk of developing Alzheimer's Disease (AD), non-AD dementia, Multiple Sclerosis (MS), Parkinson's Disease (PD) and Amyotrophic Lateral Sclerosis (ALS) was investigated. RESEARCH DESIGN AND METHODS: A retrospective cohort analysis of a medical claims dataset over a 10 year span was conducted in patients aged 60 years or older...
2024: Frontiers in Psychiatry
https://read.qxmd.com/read/38486089/solubility-of-%C3%AE-synuclein-species-in-the-l62-mouse-model-of-synucleinopathy
#37
JOURNAL ARTICLE
Karima Schwab, Mandy Magbagbeolu, Franz Theuring, Charles R Harrington, Claude M Wischik, Gernot Riedel
The accumulation of α-synuclein (α-Syn) into Lewy bodies is a hallmark of synucleinopathies, a group of neurological disorders that include Parkinson's disease (PD) and dementia with Lewy bodies (DLB). Small oligomers as well as larger fibrils of α-Syn have been suggested to induce cell toxicity leading to a degenerative loss of neurones. A richer understanding of α-Syn aggregation in disease, however, requires the identification of the different α-Syn species and the characterisation of their biochemical properties...
March 14, 2024: Scientific Reports
https://read.qxmd.com/read/38482885/eeg-spectro-spatial-covariance-patterns-related-to-phenoconversion-in-isolated-rem-sleep-behavior-disorder-and-their-longitudinal-trajectories-in-%C3%AE-synucleinopathies
#38
JOURNAL ARTICLE
Kyoungeun Park, Jung Hwan Shin, Jung-Ick Byun, El Jeong, Han-Joon Kim, Ki-Young Jung
STUDY OBJECTIVES: This study aimed to identify electroencephalographic (EEG) spectro-spatial covariance patterns associated with phenoconversion in isolated rapid eye movement sleep behavior disorder (iRBD) patients and explore their longitudinal trajectories within α-synucleinopathies. METHODS: We assessed 47 participants, including 35 iRBD patients and 12 healthy controls (HC), through baseline eye-closed resting EEGs. iRBD patients underwent follow-up EEG assessments and 18 iRBD patients converted (12 to Parkinson's disease (PD), 6 to dementia with Lewy bodies (DLB)) during follow-up...
March 14, 2024: Sleep
https://read.qxmd.com/read/38481899/outcomes-of-mechanical-thrombectomy-in-patients-with-neurological-disorders-a-national-inpatient-sample-database-analysis
#39
JOURNAL ARTICLE
Mohammad El-Ghanem, Hael Abdulrazeq, Leonardo Brasiliense, Hamza Abbad, Pedro Aguilar-Salinas, Fawaz Al-Mufti, Travis Dumont
INTRODUCTION: Mechanical thrombectomy (MT) has changed the standard of care for patients presenting with acute ischemic stroke (AIS). The window of treatment has significantly increased the number of patients who would benefit from intervention and operators may be confronted with patients harboring preexistent neurological disorders. Still, the epidemiology of patients with AIS and neurological disorders has not been established. METHODS: This is a retrospective study, which utilizes data from the National Inpatient Sample (NIS) between 2012 and 2016...
February 2024: Curēus
https://read.qxmd.com/read/38481342/differences-in-the-treatment-needs-of-patients-with-dementia-with-lewy-bodies-and-their-caregivers-and-differences-in-their-physicians-awareness-of-those-treatment-needs-according-to-the-clinical-department-visited-by-the-patients-a-subanalysis-of-an-observational
#40
JOURNAL ARTICLE
Manabu Ikeda, Shunji Toya, Yuta Manabe, Hajime Yamakage, Mamoru Hashimoto
BACKGROUND: We investigated whether the treatment needs of patients with dementia with Lewy bodies (DLB) and their caregivers, along with their attending physicians' perception of those treatment needs, differ according to the clinical department visited by the patients. METHODS: This was a subanalysis of a multicenter, cross-sectional, observational survey study. Data from the main study were classified according to the clinical department visited by the patient: psychiatric group (P-group), geriatric internal medicine group (G-group), and neurology group (N-group)...
March 14, 2024: Alzheimer's Research & Therapy
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