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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
#2
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
#3
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
#4
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
#5
JOURNAL ARTICLE
Yasushi Iwasaki
A definite diagnosis of neurodegenerative diseases is required for neuropathological examination during an autopsy. Each neurodegenerative disease has specific vulnerable regions and affected systems (system degeneration), and is typified by an accumulation of abnormal protein with the formation of characteristic morphological aggregates in the nerve and glial cells, called proteinopathy. The most common neurodegenerative diseases are tauopathy, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and Pick's disease (PiD); α-synucleinopathy, including multiple system atrophy (MSA); and TAR DNA-binding protein of 43 kDa (TDP-43) proteinopathy, including amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD)...
April 2024: Brain and Nerve, Shinkei Kenkyū No Shinpo
#6
Rowan Saloner, Adam Staffaroni, Eric Dammer, Erik C B Johnson, Emily Paolillo, Amy Wise, Hilary Heuer, Leah Forsberg, Argentina Lario Lago, Julia Webb, Jacob Vogel, Alexander Santillo, Oskar Hansson, Joel Kramer, Bruce Miller, Jingyao Li, Joseph Loureiro, Rajeev Sivasankaran, Kathleen Worringer, Nicholas Seyfried, Jennifer Yokoyama, William Seeley, Salvatore Spina, Lea Grinberg, Lawren VandeVrede, Peter Ljubenkov, Ece Bayram, Andrea Bozoki, Danielle Brushaber, Ciaran Considine, Gregory Day, Bradford Dickerson, Kimiko Domoto-Reilly, Kelley Faber, Douglas Galasko, Daniel Geschwind, Nupur Ghoshal, Neill Graff-Radford, Chadwick Hales, Lawrence Honig, Ging-Yuek Hsiung, Edward Huey, John Kornak, Walter Kremers, Maria Lapid, Suzee Lee, Irene Litvan, Corey McMillan, Mario Mendez, Toji Miyagawa, Alexander Pantelyat, Belen Pascual, Henry Paulson, Leonard Petrucelli, Peter Pressman, Eliana Ramos, Katya Rascovsky, Erik Roberson, Rodolfo Savica, Allison Snyder, A Campbell Sullivan, Carmela Tartaglia, Marijne Vandebergh, Bradley Boeve, Howie Rosen, Julio Rojas, Adam Boxer, Kaitlin Casaletto
The pathophysiological mechanisms driving disease progression of frontotemporal lobar degeneration (FTLD) and corresponding biomarkers are not fully understood. We leveraged aptamer-based proteomics (> 4,000 proteins) to identify dysregulated communities of co-expressed cerebrospinal fluid proteins in 116 adults carrying autosomal dominant FTLD mutations ( C9orf72 , GRN , MAPT ) compared to 39 noncarrier controls. Network analysis identified 31 protein co-expression modules. Proteomic signatures of genetic FTLD clinical severity included increased abundance of RNA splicing (particularly in C9orf72 and GRN ) and extracellular matrix (particularly in MAPT ) modules, as well as decreased abundance of synaptic/neuronal and autophagy modules...
March 28, 2024: Research Square
#7
Raza Haider, Brandon Shipley, Krystyna Surewicz, Michael Hinczewski, Witold K Surewicz
UNLABELLED: C-terminally phosphorylated TAR DNA-binding protein of 43 kDa (TDP-43) marks the proteinaceous inclusions that characterize a number of age-related neurodegenerative diseases, including amyotrophic lateral sclerosis, frontotemporal lobar degeneration and Alzheimer's disease. TDP-43 phosphorylation at S403/S404, and especially at S409/S410, is in fact accepted as a biomarker of proteinopathy. These residues are located within the low complexity domain (LCD), which also drives the protein's liquid-liquid phase separation (LLPS)...
March 27, 2024: bioRxiv
#8
JOURNAL ARTICLE
Sunghye Cho, Christopher A Olm, Sharon Ash, Sanjana Shellikeri, Galit Agmon, Katheryn A Q Cousins, David J Irwin, Murray Grossman, Mark Liberman, Naomi Nevler
INTRODUCTION: Screening for Alzheimer's disease neuropathologic change (ADNC) in individuals with atypical presentations is challenging but essential for clinical management. We trained automatic speech-based classifiers to distinguish frontotemporal dementia (FTD) patients with ADNC from those with frontotemporal lobar degeneration (FTLD). METHODS: We trained automatic classifiers with 99 speech features from 1 minute speech samples of 179 participants (ADNC = 36, FTLD = 60, healthy controls [HC] = 89)...
April 4, 2024: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
#9
JOURNAL ARTICLE
Methasit Jaisa-Aad, Clara Muñoz-Castro, Molly A Healey, Bradley T Hyman, Alberto Serrano-Pozo
Reactive astrogliosis accompanies the two neuropathological hallmarks of Alzheimer's disease (AD)-Aβ plaques and neurofibrillary tangles-and parallels neurodegeneration in AD and AD-related dementias (ADRD). Thus, there is growing interest in developing imaging and fluid biomarkers of reactive astrogliosis for AD/ADRD diagnosis and prognostication. Monoamine oxidase-B (MAO-B) is emerging as a target for PET imaging radiotracers of reactive astrogliosis. However, a thorough characterization of MAO-B expression in postmortem control and AD/ADRD brains is lacking...
April 3, 2024: Acta Neuropathologica
#10
JOURNAL ARTICLE
Vanina Ramognino, Thomas Fovet, Mathilde Horn, Thibaud Lebouvier, Ali Amad
BACKGROUND: Catatonia is a highly prevalent syndrome in patients presenting with major neurocognitive disorders (dementia). In this study, we aim to provide a comprehensive description of the clinical and therapeutic aspects of catatonia in patients with dementia. METHOD: This descriptive study, conducted between September 2015 and June 2022, collected data from 25 patients diagnosed with dementia, out of 143 patients treated for catatonia in our specialized psychiatry department...
March 27, 2024: Asian Journal of Psychiatry
#11
JOURNAL ARTICLE
Adam M Staffaroni, Annie L Clark, Jack C Taylor, Hilary W Heuer, Mark Sanderson-Cimino, Amy B Wise, Sreya Dhanam, Yann Cobigo, Amy Wolf, Masood Manoochehri, Leah Forsberg, Carly Mester, Katherine P Rankin, Brian S Appleby, Ece Bayram, Andrea Bozoki, David Clark, R Ryan Darby, Kimiko Domoto-Reilly, Julie A Fields, Douglas Galasko, Daniel Geschwind, Nupur Ghoshal, Neill Graff-Radford, Murray Grossman, Ging-Yuek Hsiung, Edward D Huey, David T Jones, Maria I Lapid, Irene Litvan, Joseph C Masdeu, Lauren Massimo, Mario F Mendez, Toji Miyagawa, Belen Pascual, Peter Pressman, Vijay K Ramanan, Eliana Marisa Ramos, Katya Rascovsky, Erik D Roberson, M Carmela Tartaglia, Bonnie Wong, Bruce L Miller, John Kornak, Walter Kremers, Jason Hassenstab, Joel H Kramer, Bradley F Boeve, Howard J Rosen, Adam L Boxer
IMPORTANCE: Frontotemporal lobar degeneration (FTLD) is relatively rare, behavioral and motor symptoms increase travel burden, and standard neuropsychological tests are not sensitive to early-stage disease. Remote smartphone-based cognitive assessments could mitigate these barriers to trial recruitment and success, but no such tools are validated for FTLD. OBJECTIVE: To evaluate the reliability and validity of smartphone-based cognitive measures for remote FTLD evaluations...
April 1, 2024: JAMA Network Open
#12
REVIEW
Rita Ferreira, António J Bastos-Leite
We reviewed 33 original research studies assessing brain perfusion, using consensus guidelines from a "white paper" issued by the International Society for Magnetic Resonance in Medicine Perfusion Study Group and the European Cooperation in Science and Technology Action BM1103 ("Arterial Spin Labelling Initiative in Dementia"; https://www.cost.eu/actions/BM1103/ ). The studies were published between 2011 and 2023 and included participants with subjective cognitive decline plus; neurocognitive disorders, including mild cognitive impairment (MCI), Alzheimer's disease (AD), frontotemporal lobar degeneration (FTLD), dementia with Lewy bodies (DLB) and vascular cognitive impairment (VCI); as well as schizophrenia spectrum disorders, bipolar and major depressive disorders, autism spectrum disorder, attention-deficit/hyperactivity disorder, panic disorder and alcohol use disorder...
March 27, 2024: Neuroradiology
#13
REVIEW
Ioannis Liampas, Freideriki Danga, Panagiota Kyriakoulopoulou, Vasileios Siokas, Polyxeni Stamati, Lambros Messinis, Efthimios Dardiotis, Grigorios Nasios
Functional near-infrared spectroscopy (fNIRS) is an innovative neuroimaging method that offers several advantages over other commonly used modalities. This narrative review investigated the potential contribution of this method to the study of neurodegenerative disorders. Thirty-four studies involving patients with Alzheimer's disease (AD), mild cognitive impairment (MCI), frontotemporal dementia (FTD), Parkinson's disease (PD), or amyotrophic lateral sclerosis (ALS) and healthy controls were reviewed. Overall, it was revealed that the prefrontal cortex of individuals with MCI may engage compensatory mechanisms to support declining brain functions...
March 21, 2024: Diagnostics
#14
JOURNAL ARTICLE
Time to Diagnosis and Its Predictors in Syndromes Associated With Frontotemporal Lobar Degeneration.
Ilenia Libri, Daniele Altomare, Valeria Bracca, Jasmine Rivolta, Valentina Cantoni, Irene Mattioli, Antonella Alberici, Barbara Borroni
OBJECTIVES: Frontotemporal Lobar Degeneration (FTLD) causes a heterogeneous group of neurodegenerative disorders with a wide range of clinical features. This might delay time to diagnosis. The aim of the present study is to establish time to diagnosis and its predictors in patients with FTLD-associated syndromes. DESIGN: Retrospective study. SETTING: Tertiary referral center. PARTICIPANTS: A total of 1029 patients with FTLD-associated syndromes (age: 68 [61-73] years, females: 46%) from 1999 to 2023 were included in the present study...
March 7, 2024: American Journal of Geriatric Psychiatry
#15
JOURNAL ARTICLE
S Sebastian Pineda, Hyeseung Lee, Maria J Ulloa-Navas, Raleigh M Linville, Francisco J Garcia, Kyriakitsa Galani, Erica Engelberg-Cook, Monica C Castanedes, Brent E Fitzwalter, Luc J Pregent, Mahammad E Gardashli, Michael DeTure, Diana V Vera-Garcia, Andre T S Hucke, Bjorn E Oskarsson, Melissa E Murray, Dennis W Dickson, Myriam Heiman, Veronique V Belzil, Manolis Kellis
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) share many clinical, pathological, and genetic features, but a detailed understanding of their associated transcriptional alterations across vulnerable cortical cell types is lacking. Here, we report a high-resolution, comparative single-cell molecular atlas of the human primary motor and dorsolateral prefrontal cortices and their transcriptional alterations in sporadic and familial ALS and FTLD. By integrating transcriptional and genetic information, we identify known and previously unidentified vulnerable populations in cortical layer 5 and show that ALS- and FTLD-implicated motor and spindle neurons possess a virtually indistinguishable molecular identity...
March 14, 2024: Cell
#16
JOURNAL ARTICLE
Carolina Maldonado-Díaz, Satomi Hiya, Raquel T Yokoda, Kurt Farrell, Gabriel A Marx, Justin Kauffman, Elena V Daoud, Mitzi M Gonzales, Alicia S Parker, Leyla Canbeldek, Lakshmi Shree Kulumani Mahadevan, John F Crary, Charles L White, Jamie M Walker, Timothy E Richardson
Neurodegenerative pathologies such as Alzheimer disease neuropathologic change (ADNC), Lewy body disease (LBD), limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC), and cerebrovascular disease (CVD) frequently coexist, but little is known about the exact contribution of each pathology to cognitive decline and dementia in subjects with mixed pathologies. We explored the relative cognitive impact of concurrent common and rare neurodegenerative pathologies employing multivariate logistic regression analysis adjusted for age, gender, and level of education...
March 23, 2024: Acta Neuropathologica
#17
JOURNAL ARTICLE
Dror Shir, Nick Corriveau-Lecavalier, Camilo Bermudez Noguera, Leland Barnard, Nha Trang Thu Pham, Hugo Botha, Joseph R Duffy, Heather M Clark, Rene L Utianski, David S Knopman, Ronald C Petersen, Bradley F Boeve, Melissa E Murray, Aivi T Nguyen, R Ross Reichard, Dennis W Dickson, Gregory S Day, Walter K Kremers, Neill R Graff-Radford, David T Jones, Mary M Machulda, Julie A Fields, Jennifer L Whitwell, Keith A Josephs, Jonathan Graff-Radford
BACKGROUND: Primary progressive aphasia (PPA) defines a group of neurodegenerative disorders characterised by language decline. Three PPA variants correlate with distinct underlying pathologies: semantic variant PPA (svPPA) with transactive response DNA-binding protein of 43 kD (TDP-43) proteinopathy, agrammatic variant PPA (agPPA) with tau deposition and logopenic variant PPA (lvPPA) with Alzheimer's disease (AD). Our objectives were to differentiate PPA variants using clinical and neuroimaging features, assess progression and evaluate structural MRI and a novel 18-F fluorodeoxyglucose positron emission tomography (FDG-PET) image decomposition machine learning algorithm for neuropathology prediction...
March 21, 2024: Journal of Neurology, Neurosurgery, and Psychiatry
#18
JOURNAL ARTICLE
Veronika Pak, Quadri Adewale, Danilo Bzdok, Mahsa Dadar, Yashar Zeighami, Yasser Iturria-Medina
For over a century, brain research narrative has mainly centered on neuron cells. Accordingly, most neurodegenerative studies focus on neuronal dysfunction and their selective vulnerability, while we lack comprehensive analyses of other major cell types' contribution. By unifying spatial gene expression, structural MRI, and cell deconvolution, here we describe how the human brain distribution of canonical cell types extensively predicts tissue damage in 13 neurodegenerative conditions, including early- and late-onset Alzheimer's disease, Parkinson's disease, dementia with Lewy bodies, amyotrophic lateral sclerosis, mutations in presenilin-1, and 3 clinical variants of frontotemporal lobar degeneration (behavioral variant, semantic and non-fluent primary progressive aphasia) along with associated three-repeat and four-repeat tauopathies and TDP43 proteinopathies types A and C...
March 21, 2024: ELife
#19
JOURNAL ARTICLE
Manabu Kubota, Hironobu Endo, Keisuke Takahata, Kenji Tagai, Hisaomi Suzuki, Mitsumoto Onaya, Yasunori Sano, Yasuharu Yamamoto, Shin Kurose, Kiwamu Matsuoka, Chie Seki, Hitoshi Shinotoh, Kazunori Kawamura, Ming-Rong Zhang, Yuhei Takado, Hitoshi Shimada, Makoto Higuchi
Frontotemporal dementia refers to a group of neurodegenerative disorders with diverse clinical and neuropathological features. In vivo neuropathological assessments of frontotemporal dementia at an individual level have hitherto not been successful. In this study, we aim to classify patients with frontotemporal dementia based on topologies of tau protein aggregates captured by PET with 18 F-florzolotau (aka 18 F-APN-1607 and 18 F-PM-PBB3), which allows high-contrast imaging of diverse tau fibrils in Alzheimer's disease as well as in non-Alzheimer's disease tauopathies...
2024: Brain communications
#20
JOURNAL ARTICLE
Daniel Pirici, Laurentiu Mogoanta, Daniela Adriana Ion, Samir Kumar-Singh
Neurodegenerative diseases are defined by progressive nervous system dysfunction and death of neurons. The abnormal conformation and assembly of proteins is suggested to be the most probable cause for many of these neurodegenerative disorders, leading to the accumulation of abnormally aggregated proteins, for example, amyloid β (Aβ) (Alzheimer's disease and vascular dementia), tau protein (Alzheimer's disease and frontotemporal lobar degeneration), α-synuclein (Parkinson's disease and Lewy body dementia), polyglutamine expansion diseases (Huntington disease), or prion proteins (Creutzfeldt-Jakob disease)...
2024: Advances in Neurobiology
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