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Corticobasal degeneration

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https://www.readbyqxmd.com/read/28293793/ultrasensitive-and-selective-detection-of-3-repeat-tau-seeding-activity-in-pick-disease-brain-and-cerebrospinal-fluid
#1
Eri Saijo, Bernardino Ghetti, Gianluigi Zanusso, Adrian Oblak, Jennifer L Furman, Marc I Diamond, Allison Kraus, Byron Caughey
The diagnosis and treatment of diseases involving tau-based pathology such as Alzheimer disease and certain frontotemporal dementias is hampered by the inability to detect pathological forms of tau with sufficient sensitivity, specificity and practicality. In these neurodegenerative diseases, tau accumulates in self-seeding filaments. For example, Pick disease (PiD) is associated with frontotemporal degeneration and accumulation of 3-repeat (3R) tau isoforms in filaments constituting Pick bodies. Exploiting the self-seeding activity of tau deposits, and using a 3R tau fragment as a substrate, we have developed an assay (tau RT-QuIC) that can detect tau seeds in 2 µl aliquots of PiD brain dilutions down to 10(-7)-10(-9)...
March 14, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28271184/shared-genetic-risk-between-corticobasal-degeneration-progressive-supranuclear-palsy-and-frontotemporal-dementia
#2
Jennifer S Yokoyama, Celeste M Karch, Chun C Fan, Luke W Bonham, Naomi Kouri, Owen A Ross, Rosa Rademakers, Jungsu Kim, Yunpeng Wang, Günter U Höglinger, Ulrich Müller, Raffaele Ferrari, John Hardy, Parastoo Momeni, Leo P Sugrue, Christopher P Hess, A James Barkovich, Adam L Boxer, William W Seeley, Gil D Rabinovici, Howard J Rosen, Bruce L Miller, Nicholas J Schmansky, Bruce Fischl, Bradley T Hyman, Dennis W Dickson, Gerard D Schellenberg, Ole A Andreassen, Anders M Dale, Rahul S Desikan
Corticobasal degeneration (CBD), progressive supranuclear palsy (PSP) and a subset of frontotemporal dementia (FTD) are neurodegenerative disorders characterized by tau inclusions in neurons and glia (tauopathies). Although clinical, pathological and genetic evidence suggests overlapping pathobiology between CBD, PSP, and FTD, the relationship between these disorders is still not well understood. Using summary statistics (odds ratios and p values) from large genome-wide association studies (total n = 14,286 cases and controls) and recently established genetic methods, we investigated the genetic overlap between CBD and PSP and CBD and FTD...
March 7, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28268100/intrafamilial-variable-phenotype-including-corticobasal-syndrome-in-a-family-with-p-p301l-mutation-in-the-mapt-gene-first-report-in-south-america
#3
Emilia M Gatto, Ricardo F Allegri, Gustavo Da Prat, Patricio Chrem Mendez, David S Hanna, Michael O Dorschner, Ezequiel I Surace, Cyrus P Zabetian, Ignacio F Mata
Frontotemporal lobar degeneration is a neuropathological disorder that causes a variety of clinical syndromes including frontotemporal dementia (FTD), progressive supranuclear palsy, and corticobasal syndrome (CBS). FTD associated with parkinsonism occurs frequently as a result of mutations in the C9orf72 gene and also in the genes coding for the protein associated with microtubule tau (MAPT) and progranulin (GRN) on chromosome 17 (FTDP-17). Herein, we report an Argentinean family, of Basque ancestry, with an extensive family history of behavioral variant of FTD...
February 10, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28259709/consensus-classification-of-posterior-cortical-atrophy
#4
Sebastian J Crutch, Jonathan M Schott, Gil D Rabinovici, Melissa Murray, Julie S Snowden, Wiesje M van der Flier, Bradford C Dickerson, Rik Vandenberghe, Samrah Ahmed, Thomas H Bak, Bradley F Boeve, Christopher Butler, Stefano F Cappa, Mathieu Ceccaldi, Leonardo Cruz de Souza, Bruno Dubois, Olivier Felician, Douglas Galasko, Jonathan Graff-Radford, Neill R Graff-Radford, Patrick R Hof, Pierre Krolak-Salmon, Manja Lehmann, Eloi Magnin, Mario F Mendez, Peter J Nestor, Chiadi U Onyike, Victoria S Pelak, Yolande Pijnenburg, Silvia Primativo, Martin N Rossor, Natalie S Ryan, Philip Scheltens, Timothy J Shakespeare, Aida Suárez González, David F Tang-Wai, Keir X X Yong, Maria Carrillo, Nick C Fox
INTRODUCTION: A classification framework for posterior cortical atrophy (PCA) is proposed to improve the uniformity of definition of the syndrome in a variety of research settings. METHODS: Consensus statements about PCA were developed through a detailed literature review, the formation of an international multidisciplinary working party which convened on four occasions, and a Web-based quantitative survey regarding symptom frequency and the conceptualization of PCA...
March 2, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28256434/pathologic-correlates-of-supranuclear-gaze-palsy-with-parkinsonism
#5
W R W Martin, J Hartlein, B A Racette, N Cairns, J S Perlmutter
INTRODUCTION: Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic. METHODS: We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St...
February 24, 2017: Parkinsonism & related Disorders
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
#6
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/28243754/differentiation-of-atypical-parkinson-syndromes
#7
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
https://www.readbyqxmd.com/read/28235814/differentiating-cognitive-impairment-due-to-corticobasal-degeneration-and-alzheimer-disease
#8
Gregory S Day, Tae Sung Lim, Jason Hassenstab, Alison M Goate, Elizabeth A Grant, Catherine M Roe, Nigel J Cairns, John C Morris
OBJECTIVE: To identify clinical features that reliably differentiate individuals with cognitive impairment due to corticobasal degeneration (CBD) and Alzheimer disease (AD). METHODS: Clinical features were compared between individuals with autopsy-proven CBD (n = 17) and AD (n = 16). All individuals presented with prominent cognitive complaints and were evaluated annually with semistructured interviews, detailed neurologic examinations, and neuropsychological testing...
February 24, 2017: Neurology
https://www.readbyqxmd.com/read/28207803/side-effects-induced-by-the-acute-levodopa-challenge-in-parkinson-s-disease-and-atypical-parkinsonisms
#9
Rosario Vasta, Alessandra Nicoletti, Giovanni Mostile, Valeria Dibilio, Giorgia Sciacca, Donatella Contrafatto, Calogero Edoardo Cicero, Loredana Raciti, Antonina Luca, Mario Zappia
INTRODUCTION: Acute levodopa challenge may be performed to predict levodopa chronic responsiveness. The aim of the study was to investigate frequency of side effects during the acute levodopa challenge in PD and atypical parkinsonisms. METHODS: We enrolled 34 de novo PD patients and 29 patients affected by atypical parkinsonisms (Multiple System Atrophy, MSA, n = 10; Progressive Supranuclear Palsy, PSP, n = 12 and Corticobasal Degeneration, CBD, n = 7) who underwent an acute levodopa challenge...
2017: PloS One
https://www.readbyqxmd.com/read/28205009/mixed-pathologies-including-chronic-traumatic-encephalopathy-account-for-dementia-in-retired-association-football-soccer-players
#10
Helen Ling, Huw R Morris, James W Neal, Andrew J Lees, John Hardy, Janice L Holton, Tamas Revesz, David D R Williams
In retired professional association football (soccer) players with a past history of repetitive head impacts, chronic traumatic encephalopathy (CTE) is a potential neurodegenerative cause of dementia and motor impairments. From 1980 to 2010, 14 retired footballers with dementia were followed up regularly until death. Their clinical data, playing career, and concussion history were prospectively collected. Next-of-kin provided consent for six to have post-mortem brain examination. Of the 14 male participants, 13 were professional and 1 was a committed amateur...
March 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28126506/-other-causes-of-parkinsonism
#11
Christine Tranchant
Numerous causes may induce parkinsonism, and in case of Parkinsonism, l-dopa resistance, associated neurological signs, early falls, early dysautonomia or early cognitive disorders are the main red flags which should lead to exclude Parkinson's disease. Drug-induced parkinsonism and in young people, Wilson disease, should always be searched. Lower limbs parkinsonism is often due to vascular lesions. Multiple systemic atrophy, supranuclear palsy, Lewy body disease and the rarer corticobasal degeneration, each have specific clinical signs...
January 23, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28100725/genome-wide-association-study-identifies-mapt-locus-influencing-human-plasma-tau-levels
#12
Jason Chen, Jin-Tai Yu, Kevin Wojta, Hui-Fu Wang, Henrik Zetterberg, Kaj Blennow, Jennifer S Yokoyama, Michael W Weiner, Joel H Kramer, Howard Rosen, Bruce L Miller, Giovanni Coppola, Adam L Boxer
OBJECTIVE: To identify genetic loci associated with plasma tau concentrations in healthy elders and individuals with Alzheimer disease. METHODS: Four hundred sixty-three non-Hispanic white individuals exceeding quality control criteria were included from the Alzheimer's Disease Neuroimaging Initiative (ADNI-1) cohort. Association of plasma tau with genetic polymorphisms was performed with a linear regression model. Significant associations were validated in an independent replication cohort consisting of 431 healthy elders or individuals with mild cognitive impairment recruited from the University of California, San Francisco Memory and Aging Center...
February 14, 2017: Neurology
https://www.readbyqxmd.com/read/28087578/distinct-binding-of-pet-ligands-pbb3-and-av-1451-to-tau-fibril-strains-in-neurodegenerative-tauopathies
#13
Maiko Ono, Naruhiko Sahara, Katsushi Kumata, Bin Ji, Ruiqing Ni, Shunsuke Koga, Dennis W Dickson, John Q Trojanowski, Virginia M-Y Lee, Mari Yoshida, Isao Hozumi, Yasumasa Yoshiyama, John C van Swieten, Agneta Nordberg, Tetsuya Suhara, Ming-Rong Zhang, Makoto Higuchi
Diverse neurodegenerative disorders are characterized by deposition of tau fibrils composed of conformers (i.e. strains) unique to each illness. The development of tau imaging agents has enabled visualization of tau lesions in tauopathy patients, but the modes of their binding to different tau strains remain elusive. Here we compared binding of tau positron emission tomography ligands, PBB3 and AV-1451, by fluorescence, autoradiography and homogenate binding assays with homologous and heterologous blockades using tauopathy brain samples...
January 12, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28077166/threonine-175-a-novel-pathological-phosphorylation-site-on-tau-protein-linked-to-multiple-tauopathies
#14
Alexander J Moszczynski, Wencheng Yang, Robert Hammond, Lee Cyn Ang, Michael J Strong
Microtubule associated protein tau (tau) deposition is associated with a spectrum of neurodegenerative diseases collectively termed tauopathies. We have previously shown that amyotrophic lateral sclerosis (ALS) with cognitive impairment (ALSci) is associated with tau phosphorylation at Thr(175) and that this leads to activation of GSK3β which then induces phosphorylation at tau Thr(231). This latter step leads to dissociation of tau from microtubules and pathological tau fibril formation. To determine the extent to which this pathway is unique to ALS, we have investigated the expression of pThr(175) tau and pThr(231) tau across a range of frontotemporal degenerations...
January 11, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27999880/-pathomechanisms-and-clinical-aspects-of-frontotemporal-lobar-degeneration
#15
K Bürger, T Arzberger, J Stephan, J Levin, D Edbauer
BACKGROUND: Frontotemporal lobar degeneration (FTLD) includes a spectrum of heterogeneous clinical and neuropathological diseases. In a strict sense this includes the behavioral variant of frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) and both variants can be associated with amyotrophic lateral sclerosis (FTD-ALS). In a broader sense FTLD also includes progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). In recent years the strong genetic component of FTLD has become increasingly clear...
February 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/27997993/longitudinal-imaging-reveals-subhippocampal-dynamics-in-glutamate-levels-associated-with-histopathologic-events-in-a-mouse-model-of-tauopathy-and-healthy-mice
#16
Rachelle Crescenzi, Catherine DeBrosse, Ravi P R Nanga, Matthew D Byrne, Guruprasad Krishnamoorthy, Kevin D'Aquilla, Hari Nath, Knashawn H Morales, Michiyo Iba, Hari Hariharan, Virginia M Y Lee, John A Detre, Ravinder Reddy
Tauopathies are neurodegenerative disorders characterized by abnormal intracellular aggregates of tau protein, and include Alzheimer's disease, corticobasal degeneration, frontotemporal dementia, and traumatic brain injury. Glutamate metabolism is altered in neurodegenerative disorders manifesting in higher or lower concentrations of glutamate, its transporters or receptors. Previously, glutamate chemical exchange saturation transfer (GluCEST) magnetic resonance imaging (MRI) demonstrated that glutamate levels are reduced in regions of synapse loss in the hippocampus of a mouse model of late-stage tauopathy...
March 2017: Hippocampus
https://www.readbyqxmd.com/read/27987553/molecular-imaging-of-extrapyramidal-movement-disorders
#17
REVIEW
Kirk A Frey
Extrapyramidal movement disorders including Parkinson disease, multiple systems atrophy, progressive supranuclear palsy, and corticobasal degeneration are neurodegenerative syndromes with distinct neuropathological changes, indicating differing underlying etiologies. Clinical features that may distinguish among these conditions are often absent, particularly early after the onset of symptoms. Therapy is presently limited, and there are no established disease-modifying or neuroprotective interventions. Advances in therapeutics will depend on the early and accurate diagnostic classification of patients...
January 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/27913406/presymptomatic-anterior-frontal-involvement-in-corticobasal-degeneration
#18
Christopher Kobylecki, David M Mann
No abstract text is available yet for this article.
December 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27911827/tau-prions-from-alzheimer-s-disease-and-chronic-traumatic-encephalopathy-patients-propagate-in-cultured-cells
#19
Amanda L Woerman, Atsushi Aoyagi, Smita Patel, Sabeen A Kazmi, Iryna Lobach, Lea T Grinberg, Ann C McKee, William W Seeley, Steven H Olson, Stanley B Prusiner
Tau prions are thought to aggregate in the central nervous system, resulting in neurodegeneration. Among the tauopathies, Alzheimer's disease (AD) is the most common, whereas argyrophilic grain disease (AGD), corticobasal degeneration (CBD), chronic traumatic encephalopathy (CTE), Pick's disease (PiD), and progressive supranuclear palsy (PSP) are less prevalent. Brain extracts from deceased individuals with PiD, a neurodegenerative disorder characterized by three-repeat (3R) tau prions, were used to infect HEK293T cells expressing 3R tau fused to yellow fluorescent protein (YFP)...
December 13, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27842611/tau-pathology-in-aged-cynomolgus-monkeys-is-progressive-supranuclear-palsy-corticobasal-degeneration-but-not-alzheimer-disease-like-ultrastructural-mapping-of-tau-by-edx
#20
Toshiki Uchihara, Kentaro Endo, Hiromi Kondo, Sachi Okabayashi, Nobuhiro Shimozawa, Yasuhiro Yasutomi, Eijiro Adachi, Nobuyuki Kimura
Concomitant deposition of amyloid -beta protein (Aβ) and neuronal tau as neurofibrillary tangles in the human brain is a hallmark of Alzheimer disease (AD). Because these deposits increase during normal aging, it has been proposed that aging brains may also undergo AD-like changes. To investigate the neuropathological changes that occur in the aging primate brain, we examined 21 brains of cynomolgus monkeys (7-36 years old) for Aβ- and tau-positive lesions. We found, 1) extensive deposition of Aβ in brains of cynomolgus monkeys over 25 years of age, 2) selective deposition of 4-repeat tau as pretangles in neurons, and as coiled body-like structures in oligodendroglia-like cells and astrocytes, 3) preferential distribution of tau in the basal ganglia and neocortex rather than the hippocampus, and 4) age-associated increases in 30-34 kDa AT8- and RD4-positive tau fragments in sarkosyl-insoluble fractions...
November 14, 2016: Acta Neuropathologica Communications
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