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JOURNAL ARTICLE
Yaoru Li, Ziying Yang, Yanxin Zhang, Fang Liu, Jing Xu, Yaping Meng, Gebeili Xing, Xuqin Ruan, Jun Sun, Nan Zhang
BACKGROUND: Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) account for the vast majority of neurodegenerative dementias. AD and FTLD have different clinical phenotypes with a genetic overlap between them and other dementias. OBJECTIVE: This study aimed to identify the genetic spectrum of sporadic AD and FTLD in the Chinese population. METHODS: A total of 74 sporadic AD and 29 sporadic FTLD participants were recruited...
April 29, 2024: Journal of Alzheimer's Disease: JAD
#2
JOURNAL ARTICLE
K A Kolmakova, V Yu Lobzin, A Yu Emelin, I V Litvinenko
OBJECTIVE: To study the severity and localization of dilated perivascular spaces (DPVS), the levels of protein markers of amyloidosis and neurodegeneration in the cerebrospinal fluid (CSF) at different daily blood pressure (BP) profiles in patients with Alzheimer's disease (AD) and other types of cognitive impairment. MATERIAL AND METHODS: A total of 119 people, aged 53 to 92 years, including 55 patients with AD, 27 patients with vascular cognitive disorders (VCD), 19 patients with frontotemporal degeneration (FTD)...
2024: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
#3
REVIEW
Francesca R Buccellato, Marianna D'Anca, Gianluca Martino Tartaglia, Massimo Del Fabbro, Daniela Galimberti
INTRODUCTION: Frontotemporal dementia (FTD) includes a group of neurodegenerative diseases characterized clinically by behavioral disturbances and by neurodegeneration of brain anterior temporal and frontal lobes, leading to atrophy. Apart from symptomatic treatments, there is, at present, no disease-modifying cure for FTD. AREAS COVERED: Three main mutations are known as causes of familial FTD, and large consortia have studied carriers of mutations, also in preclinical Phases...
April 30, 2024: Expert Opinion on Investigational Drugs
#4
JOURNAL ARTICLE
Christopher B Morrow, Gregory M Pontone
The following commentary discusses a review by Cressot et al. entitled: 'Psychosis in Neurodegenerative Dementias: A Systematic Comparative Review'. The authors describe the epidemiology and phenomenology of psychosis across neurodegenerative dementias. Dementia with Lewy bodies had the highest reported prevalence of psychosis at 74% followed by Alzheimer's disease, 54% and frontotemporal degeneration, 42% . Detailed characterization of psychosis shows differences in the types of hallucinations and delusions by dementia type...
April 20, 2024: Journal of Alzheimer's Disease: JAD
#5
Coralie Cressot, Agathe Vrillon, Matthieu Lilamand, Hélène Francisque, Aurélie Méauzoone, Claire Hourregue, Julien Dumurgier, Emeline Marlinge, Claire Paquet, Emmanuel Cognat
BACKGROUND: Psychosis, characterized by delusions and/or hallucinations, is frequently observed during the progression of Alzheimer's disease (AD) and other neurodegenerative dementias (ND) (i.e., dementia with Lewy bodies (DLB), and frontotemporal dementia (FTD)) and cause diagnostic and management difficulties. OBJECTIVE: This review aims at presenting a concise and up-to-date overview of psychotic symptoms that occur in patients with ND with a comparative approach...
April 26, 2024: Journal of Alzheimer's Disease: JAD
#6
JOURNAL ARTICLE
Laurel Officer, Carmel Armon, Paul Barkhaus, Morgan Beauchamp, Michael Benatar, Tulio Bertorini, Robert Bowser, Mark Bromberg, Andrew Brown, Olimpia Mihaela Carbunar, Gregory T Carter, Jesse Crayle, Keelie Denson, Eva Feldman, Timothy Fullam, Terry Heiman-Patterson, Carlayne Jackson, Sartaj Jhooty, Danelle Levinson, Xiaoyan Li, Alexandra Linares, Elise Mallon, Javier Mascias Cadavid, Christopher Mcdermott, Tasnim Mushannen, Lyle Ostrow, Ronak Patel, Gary Pattee, Dylan Ratner, Yuyao Sun, John Sladky, Paul Wicks, Richard Bedlack
Spurred by patient interest, ALSUntangled herein examines the potential of the Portable Neuromodulation Stimulator (PoNS™) in treating amyotrophic lateral sclerosis (ALS). The PoNS™ device, FDA-approved for the treatment of gait deficits in adult patients with multiple sclerosis, utilizes translingual neurostimulation to stimulate trigeminal and facial nerves via the tongue, aiming to induce neuroplastic changes. While there are early, promising data for PoNS treatment to improve gait and balance in multiple sclerosis, stroke, and traumatic brain injury, no pre-clinical or clinical studies have been performed in ALS...
April 26, 2024: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
#7
JOURNAL ARTICLE
Lisanne M Jenkins, Ashley Heywood, Sonya Gupta, Maryam Kouchakidivkolaei, Jaiashre Sridhar, Emily Rogalski, Sandra Weintraub, Karteek Popuri, Howard Rosen, Lei Wang
Disinhibition is one of the most distressing and difficult to treat neuropsychiatric symptoms of dementia. It involves socially inappropriate behaviours, such as hypersexual comments, inappropriate approaching of strangers and excessive jocularity. Disinhibition occurs in multiple dementia syndromes, including behavioural variant frontotemporal dementia, and dementia of the Alzheimer's type. Morphometric similarity networks are a relatively new method for examining brain structure and can be used to calculate measures of network integrity on large scale brain networks and subnetworks such as the salience network and cognitive control network...
2024: Brain communications
#8
REVIEW
Madia Lozupone, Vittorio Dibello, Antonio Daniele, Vincenzo Solfrizzi, Emanuela Resta, Francesco Panza
INTRODUCTION: Tauopathies are a spectrum of clinicopathological neurodegenerative disorders with increased aggregates included in glia and/or neurons of hyperphosphorylated insoluble tau protein, a microtubule-associated protein. Progressive supranuclear palsy (PSP) is an atypical dopaminergic-resistant parkinsonian syndrome, considered as a primary tauopathy with possible alteration of tau isoform ratio, and tau accumulations characterized by 4 R tau species as the main neuropathological lesions...
April 23, 2024: Expert Opinion on Pharmacotherapy
#9
Owen Dando, Robert McGeachan, Jamie McQueen, Paul Baxter, Nathan Rockley, Hannah McAlister, Adharsh Prasad, Xin He, Declan King, Jamie Rose, Phillip B Jones, Jane Tulloch, Siddharthan Chandran, Colin Smith, Giles Hardingham, Tara L Spires-Jones
Mutations in the MAPT gene encoding tau protein can cause autosomal dominant neurodegenerative tauopathies including frontotemporal dementia (often with Parkinsonism). In Alzheimer's disease, the most common tauopathy, synapse loss is the strongest pathological correlate of cognitive decline. Recently, PET imaging with synaptic tracers revealed clinically relevant loss of synapses in primary tauopathies; however, the molecular mechanisms leading to synapse degeneration in primary tauopathies remain largely unknown...
April 12, 2024: medRxiv
#10
Brandon-Joe Cilia, Dhamidhu Eratne, Cassandra Wannan, Charles Malpas, Shorena Janelidze, Oskar Hansson, Ian Everall, Chad Bousman, Naveen Thomas, Alexander F Santillo, Dennis Velakoulis, Christos Pantelis
BACKGROUND AND HYPOTHESIS: Around 30% of people with schizophrenia are refractory to antipsychotic treatment (treatment-resistant schizophrenia; TRS). While abnormal structural neuroimaging findings, in particular volume and thickness reductions, are often observed in schizophrenia, it is anticipated that biomarkers of neuronal injury like neurofilament light chain protein (NfL) can improve our understanding of the pathological basis underlying schizophrenia. The current study aimed to determine whether people with TRS demonstrate different associations between plasma NfL levels and regional cortical thickness reductions compared with controls...
April 8, 2024: medRxiv
#11
Daniel T Ohm, Sharon X Xie, Noah Capp, Sanaz Arezoumandan, Katheryn A Q Cousins, Katya Rascovsky, David A Wolk, Vivianna M Van Deerlin, Edward B Lee, Corey T McMillan, David J Irwin
Behavioral variant frontotemporal dementia (bvFTD) is a clinical syndrome primarily caused by either tau (bvFTD-tau) or TDP-43 (bvFTD-TDP) proteinopathies. We previously found lower cortical layers and dorsolateral regions accumulate greater tau than TDP-43 pathology; however, patterns of laminar neurodegeneration across diverse cytoarchitecture in bvFTD is understudied. We hypothesized that bvFTD-tau and bvFTD-TDP have distinct laminar distributions of pyramidal neurodegeneration along cortical gradients, a topologic order of cytoarchitectonic subregions based on increasing pyramidal density and laminar differentiation...
April 9, 2024: bioRxiv
#12
JOURNAL ARTICLE
Evan Udine, Mariely DeJesus-Hernandez, Shulan Tian, Sofia Pereira das Neves, Richard Crook, NiCole A Finch, Matthew C Baker, Cyril Pottier, Neill R Graff-Radford, Bradley F Boeve, Ronald C Petersen, David S Knopman, Keith A Josephs, Björn Oskarsson, Sandro Da Mesquita, Leonard Petrucelli, Tania F Gendron, Dennis W Dickson, Rosa Rademakers, Marka van Blitterswijk
The most prominent genetic cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) is a repeat expansion in the gene C9orf72. Importantly, the transcriptomic consequences of the C9orf72 repeat expansion remain largely unclear. Here, we used short-read RNA sequencing (RNAseq) to profile the cerebellar transcriptome, detecting alterations in patients with a C9orf72 repeat expansion. We focused on the cerebellum, since key C9orf72-related pathologies are abundant in this neuroanatomical region, yet TDP-43 pathology and neuronal loss are minimal...
April 19, 2024: Acta Neuropathologica
#13
Marijne Vandebergh, Eliana Marisa Ramos, Nick Corriveau-Lecavalier, Vijay K Ramanan, John Kornak, Carly Mester, Tyler Kolander, Danielle Brushaber, Adam M Staffaroni, Daniel Geschwind, Amy Wolf, Kejal Kantarci, Tania F Gendron, Leonard Petrucelli, Marleen Van den Broeck, Sarah Wynants, Matthew C Baker, Sergi Borrego-Écija, Brian Appleby, Sami Barmada, Andrea Bozoki, David Clark, R Ryan Darby, Bradford C Dickerson, Kimiko Domoto-Reilly, Julie A Fields, Douglas R Galasko, Nupur Ghoshal, Neill Graff-Radford, Ian M Grant, Lawrence S Honig, Ging-Yuek Robin Hsiung, Edward D Huey, David Irwin, David S Knopman, Justin Y Kwan, Gabriel C Léger, Irene Litvan, Joseph C Masdeu, Mario F Mendez, Chiadi Onyike, Belen Pascual, Peter Pressman, Aaron Ritter, Erik D Roberson, Allison Snyder, Anna Campbell Sullivan, M Carmela Tartaglia, Dylan Wint, Hilary W Heuer, Leah K Forsberg, Adam L Boxer, Howard J Rosen, Bradley F Boeve, Rosa Rademakers
BACKGROUND AND OBJECTIVES: TMEM106B has been proposed as a modifier of disease risk in FTLD-TDP, particularly in GRN mutation carriers. Furthermore, TMEM106B has been investigated as a disease modifier in the context of healthy aging and across multiple neurodegenerative diseases. The objective of this study is to evaluate and compare the effect of TMEM106B on gray matter volume and cognition in each of the common genetic FTD groups and in sporadic FTD patients. METHODS: Participants were enrolled through the ARTFL/LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) study, which includes symptomatic and presymptomatic individuals with a pathogenic mutation in C9orf72, GRN, MAPT, VCP, TBK1, TARDBP, symptomatic non-mutation carriers, and non-carrier family controls...
April 5, 2024: medRxiv
#14
JOURNAL ARTICLE
Rebecca R Valentino, William J Scotton, Shanu F Roemer, Tammaryn Lashley, Michael G Heckman, Maryam Shoai, Alejandro Martinez-Carrasco, Nicole Tamvaka, Ronald L Walton, Matthew C Baker, Hannah L Macpherson, Raquel Real, Alexandra I Soto-Beasley, Kin Mok, Tamas Revesz, Elizabeth A Christopher, Michael DeTure, William W Seeley, Edward B Lee, Matthew P Frosch, Laura Molina-Porcel, Tamar Gefen, Javier Redding-Ochoa, Bernardino Ghetti, Andrew C Robinson, Christopher Kobylecki, James B Rowe, Thomas G Beach, Andrew F Teich, Julia L Keith, Istvan Bodi, Glenda M Halliday, Marla Gearing, Thomas Arzberger, Christopher M Morris, Charles L White, Naguib Mechawar, Susana Boluda, Ian R MacKenzie, Catriona McLean, Matthew D Cykowski, Shih-Hsiu J Wang, Caroline Graff, Rashed M Nagra, Gabor G Kovacs, Giorgio Giaccone, Manuela Neumann, Lee-Cyn Ang, Agostinho Carvalho, Huw R Morris, Rosa Rademakers, John A Hardy, Dennis W Dickson, Jonathan D Rohrer, Owen A Ross
BACKGROUND: Pick's disease is a rare and predominantly sporadic form of frontotemporal dementia that is classified as a primary tauopathy. Pick's disease is pathologically defined by the presence in the frontal and temporal lobes of Pick bodies, composed of hyperphosphorylated, three-repeat tau protein, encoded by the MAPT gene. MAPT has two distinct haplotypes, H1 and H2; the MAPT H1 haplotype is the major genetic risk factor for four-repeat tauopathies (eg, progressive supranuclear palsy and corticobasal degeneration), and the MAPT H2 haplotype is protective for these disorders...
May 2024: Lancet Neurology
#15
JOURNAL ARTICLE
(no author information available yet)
No abstract text is available yet for this article.
May 2024: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
#16
JOURNAL ARTICLE
Zhengdong Xu, Jianxin Zhang, Jiaxing Tang, Yehong Gong, Yu Zou, Qingwen Zhang
The aggregation of transactive response deoxyribonucleic acid (DNA) binding protein of 43 kDa (TDP-43) into ubiquitin-positive inclusions is closely associated with amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration, and chronic traumatic encephalopathy. The 370-375 fragment of TDP-43 (370 GNNSYS375 , TDP-43370-375 ), the amyloidogenic hexapeptides, can be prone to forming pathogenic amyloid fibrils with the characteristic of steric zippers. Previous experiments reported the ALS-associated mutation, serine 375 substituted by glycine (S375G) is linked to early onset disease and protein aggregation of TDP-43...
March 29, 2024: Biophysical Chemistry
#17
JOURNAL ARTICLE
Peter T Nelson, David W Fardo, Xian Wu, Khine Zin Aung, Matthew D Cykowski, Yuriko Katsumata
Limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC) is detectable at autopsy in more than one-third of people beyond age 85 years and is robustly associated with dementia independent of other pathologies. Although LATE-NC has a large impact on public health, there remain uncertainties about the underlying biologic mechanisms. Here, we review the literature from human studies that may shed light on pathogenetic mechanisms. It is increasingly clear that certain combinations of pathologic changes tend to coexist in aging brains...
April 13, 2024: Journal of Neuropathology and Experimental Neurology
#18
JOURNAL ARTICLE
Yoshinori Tanaka, Lina Kozuma, Hirotsugu Hino, Kosuke Takeya, Masumi Eto
(Macro)autophagy is a cellular degradation system for unnecessary materials, such as aggregate-prone TDP-43, a central molecule in neurodegenerative diseases including amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Abemaciclib (Abe) and vacuolin-1 (Vac) treatments are known to induce vacuoles characterized by an autophagosome and a lysosome component, suggesting that they facilitate autophagosome-lysosome fusion. However, it remains unknown whether Abe and Vac suppress the accumulation of aggregate-prone TDP-43 by accelerating autophagic flux...
July 2024: Biochemistry and Biophysics Reports
#19
JOURNAL ARTICLE
Emma Rhodes, Sebleh Alfa, Hannah A Jin, Lauren Massimo, Lauren Elman, Defne Amado, Michael Baer, Colin Quinn, Corey T McMillan
OBJECTIVE: Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative condition featuring variable degrees of motor and cognitive impairment. We assessed the impact of specific, empirically derived occupational skills and requirements on cognitive and motor functioning in ALS. METHODS: Individuals with ALS (n = 150) were recruited from the University of Pennsylvania's Comprehensive ALS Clinic. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) measured cognition, and the Penn Upper Motor Neuron (PUMNS) and ALS Functional Rating Scales (ALSFRS-R) measured motor symptoms...
April 9, 2024: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
#20
JOURNAL ARTICLE
Evelyn O Talbott, Angela M Malek, Vincent C Arena, Fan Wu, Kristen Steffes, Ravi K Sharma, Jeanine Buchanich, Judith R Rager, Todd Bear, Caroline A Hoffman, David Lacomis, Chris Donnelly, Jocelyn Mauna, John E Vena
Objective: Neurotoxic chemicals are suggested in the etiology of amyotrophic lateral sclerosis (ALS). We examined the association of environmental and occupational risk factors including persistent organochlorine pesticides (OCPs) and ALS risk among cases from the Centers for Disease Control and Prevention National ALS Registry and age, sex, and county-matched controls. Methods: Participants completed a risk factor survey and provided a blood sample for OCP measurement. ALS cases were confirmed through the Registry...
April 9, 2024: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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