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Corticobasal degeneration

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https://www.readbyqxmd.com/read/29932266/eanm-ean-recommendations-for-the-use-of-brain-18-f-fluorodeoxyglucose-positron-emission-tomography-fdg-pet-in-neurodegenerative-cognitive-impairment-and-dementia-delphi-consensus
#1
Flavio Nobili, Javier Arbizu, Femke Bouwman, Alexander Drzezga, Federica Agosta, Peter Nestor, Zuzana Walker, Marina Boccardi
BACKGROUND: Recommendations for using FDG-PET to support the diagnosis of dementing neurodegenerative disorders are sparse and poorly structured. METHODS: We defined 21 questions on diagnostic issues and on semi-automated analysis to assist visual reading. Literature was reviewed to assess study design, risk of bias, inconsistency, imprecision, indirectness and effect size. Critical outcomes were sensitivity, specificity, accuracy, positive/negative predictive value, area under the receiving operating characteristic curve, and positive/negative likelihood ratio of FDG-PET in detecting the target conditions...
June 22, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29931723/cognitive-reserve-maps-the-core-loci-of-neurodegeneration-in-corticobasal-degeneration
#2
Valeria Isella, Stefano Giuseppe Grisanti, Francesca Ferri, Sabrina Morzenti, Cinzia Crivellaro, Monica Musarra, Carlo Ferrarese
BACKGROUND: Cognitively stimulating life experiences and activities are deemed to moderate the clinical impact of brain damage progressively building a neural and cognitive reserve (CR). CR has been studied extensively in various neurodegenerative disorders, but not in Corticobasal degeneration (CBD). METHODS: Using SPM8 we correlated years of education, as determinant of cognitive reserve, with tracer uptake on FDG-PET, as marker of neurodegeneration, in 35 patients with various phenotypes of CBD, including a cognitive-motor composite score or symptoms duration as covariates for controlling disease stage...
June 22, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29926172/corticobasal-degeneration-with-tdp-43-pathology-presenting-with-progressive-supranuclear-palsy-syndrome-a-distinct-clinicopathologic-subtype
#3
Shunsuke Koga, Naomi Kouri, Ronald L Walton, Mark T W Ebbert, Keith A Josephs, Irene Litvan, Neill Graff-Radford, J Eric Ahlskog, Ryan J Uitti, Jay A van Gerpen, Bradley F Boeve, Adam Parks, Owen A Ross, Dennis W Dickson
Corticobasal degeneration (CBD) is a clinically heterogeneous tauopathy, which has overlapping clinicopathologic and genetic characteristics with progressive supranuclear palsy (PSP). This study aimed to elucidate whether transactive response DNA-binding protein of 43 kDa (TDP-43) pathology contributes to clinicopathologic heterogeneity of CBD. Paraffin-embedded sections of the midbrain, pons, subthalamic nucleus, and basal forebrain from 187 autopsy-confirmed CBD cases were screened with immunohistochemistry for phospho-TDP-43...
June 20, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29925998/astrocytic-tau-pathologies-in-argyrophilic-grain-disease-and-related-four-repeat-tauopathies
#4
REVIEW
Chikako Ikeda, Osamu Yokota, Tomoko Miki, Shintaro Takenoshita, Hideki Ishizu, Seishi Terada, Norihito Yamada
Neurodegenerative diseases in which tau accumulation plays a cardinal role in the pathogenic process are called tauopathies, and when tau isoforms having four repeats of the microtubule binding sites, four-repeat tau, are selectively accumulated as pathological hallmarks, the term four-repeat tauopathy is used. The major four-repeat tauopathies are progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and argyrophilic grain disease (AGD). Historically, neuronal cytopathologies, e.g., neurofibrillary tangles and ballooned neurons, were emphasized as characteristic lesions in PSP and CBD...
June 2018: Acta Medica Okayama
https://www.readbyqxmd.com/read/29903629/magnetic-resonance-imaging-in-dementia
#5
L Raposo Rodríguez, D J Tovar Salazar, N Fernández García, L Pastor Hernández, Ó Fernández Guinea
OBJECTIVE: To describe and illustrate the key findings on structural magnetic resonance imaging (MRI) in the most common dementias of neurodegenerative origin: Alzheimer's disease, vascular dementia, dementia with Lewy bodies, variants of frontotemporal dementia, progressive supranuclear palsy, variants of multiple system atrophy, Parkinson dementia, and corticobasal degeneration. CONCLUSION: Today the role of MRI is no longer limited to ruling out underlying causes of cognitive deterioration...
June 11, 2018: Radiología
https://www.readbyqxmd.com/read/29898972/diffuse-lewy-body-disease-manifesting-as-corticobasal-syndrome-a-rare-form-of-lewy-body-disease
#6
Koji Kasanuki, Keith A Josephs, Tanis J Ferman, Melissa E Murray, Shunsuke Koga, Takuya Konno, Nobutaka Sakae, Adam Parks, Ryan J Uitti, Jay A Van Gerpen, Neill R Graff-Radford, Zbigniew K Wszolek, Dennis W Dickson
OBJECTIVE: To describe clinical and pathologic characteristics of diffuse Lewy body disease (DLBD) manifesting as corticobasal syndrome (CBS). METHODS: In 523 autopsy-confirmed cases of DLBD, we identified 11 patients diagnosed with CBS. For comparison, we studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). Given previous studies suggesting the importance of pathology in peri-Rolandic cortices in CBS, we used digital pathology to count Lewy bodies and to quantify intracytoplasmic and neuritic α-synuclein and phospho-tau burden in the motor cortex...
June 13, 2018: Neurology
https://www.readbyqxmd.com/read/29891013/sequential-stages-and-distribution-patterns-of-aging-related-tau-astrogliopathy-artag-in-the-human-brain
#7
Gabor G Kovacs, Sharon X Xie, John L Robinson, Edward B Lee, Douglas H Smith, Theresa Schuck, Virginia M-Y Lee, John Q Trojanowski
Aging-related tau astrogliopathy (ARTAG) describes tau pathology in astrocytes in different locations and anatomical regions. In the present study we addressed the question of whether sequential distribution patterns can be recognized for ARTAG or astroglial tau pathologies in both primary FTLD-tauopathies and non-FTLD-tauopathy cases. By evaluating 687 postmortem brains with diverse disorders we identified ARTAG in 455. We evaluated frequencies and hierarchical clustering of anatomical involvement and used conditional probability and logistic regression to model the sequential distribution of ARTAG and astroglial tau pathologies across different brain regions...
June 11, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29886532/purification-and-fibrillation-of-recombinant-human-amyloid-%C3%AE-prion-protein-and-tau-under-native-conditions
#8
Alexander Sandberg, Sofie Nyström
Protein misfolding, aggregation, and amyloid formation is involved in a large number of diseases. Recombinantly expressed proteins to study the amyloid fibril formation process are important for mechanistic studies. We here report protocols for production, purification, and fibrillation of three different proteins commonly found in cerebral amyloid; Aβ and Tau found in Alzheimer's disease, Chronic traumatic brain injury, Corticobasal degeneration, and Progressive Supranuclear Palsy and human prion protein found in Creutzfeldt-Jakob's disease...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29882467/a-progressive-breakdown-of-the-body-in-space
#9
Evie Kourtidou, Dimitrios Kasselimis, George Makrydakis, Lina Chatziantoniou, Andreas Kyrozis, Ioannis Evdokimidis, Constantin Potagas
A 74 year-old woman (MD), free of previous neurological history, presented with difficulty in handling cutlery, clothes, writing with what was initially described as an atypical apraxia in acts related to space. Initial neurological evaluation revealed mixed, asymmetric pyramidal, and extrapyramidal semiology. Νeuropsychological testing revealed dressing and constructional deficits, ideomotor apraxia and signs of executive dysfunction in absence of memory, language, and visual perception pathology. The final diagnosis was that of a corticobasal degeneration, where the rare occurrence of a progressively emerging syndrome of self-management loss within peripersonal space is observed...
June 8, 2018: Neurocase
https://www.readbyqxmd.com/read/29878075/neurodegenerative-disease-concomitant-proteinopathies-are-prevalent-age-related-and-apoe4-associated
#10
John L Robinson, Edward B Lee, Sharon X Xie, Lior Rennert, EunRan Suh, Colin Bredenberg, Carrie Caswell, Vivianna M Van Deerlin, Ning Yan, Ahmed Yousef, Howard I Hurtig, Andrew Siderowf, Murray Grossman, Corey T McMillan, Bruce Miller, John E Duda, David J Irwin, David Wolk, Lauren Elman, Leo McCluskey, Alice Chen-Plotkin, Daniel Weintraub, Steven E Arnold, Johannes Brettschneider, Virginia M-Y Lee, John Q Trojanowski
Lewy bodies commonly occur in Alzheimer's disease, and Alzheimer's disease pathology is frequent in Lewy body diseases, but the burden of co-pathologies across neurodegenerative diseases is unknown. We assessed the extent of tau, amyloid-β, α-synuclein and TDP-43 proteinopathies in 766 autopsied individuals representing a broad spectrum of clinical neurodegenerative disease. We interrogated pathological Alzheimer's disease (n = 247); other tauopathies (n = 95) including Pick's disease, corticobasal disease and progressive supranuclear palsy; the synucleinopathies (n = 164) including multiple system atrophy and Lewy body disease; the TDP-43 proteinopathies (n = 188) including frontotemporal lobar degeneration with TDP-43 inclusions and amyotrophic lateral sclerosis; and a minimal pathology group (n = 72)...
June 5, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29799310/clinical-and-imaging-progression-over-10-years-in-a-patient-with-primary-progressive-apraxia-of-speech-and-autopsy-confirmed-corticobasal-degeneration
#11
Katerina A Tetzloff, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Sarah M Boland, Rene L Utianski, Hugo Botha, Matthew L Senjem, Christopher G Schwarz, Keith A Josephs, Jennifer L Whitwell
Primary progressive apraxia of speech (PPAOS) is a neurodegenerative disorder in which AOS is the sole presenting complaint. We report clinical and neuroimaging data spanning 10 years from disease onset-to-death in a 49 year-old male PPAOS patient, DY, who died with corticobasal degeneration. He presented with AOS with normal neuroimaging. Abnormalities in the caudate nucleus, supplementary motor area, cingulate, insula, and Broca's area were observed after five years, with involvement of motor cortex and development of agrammatism, Parkinsonism, and dysarthria three years later...
May 25, 2018: Neurocase
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#12
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29755341/plasma-biomarkers-differentiate-parkinson-s-disease-from-atypical-parkinsonism-syndromes
#13
Chin-Hsien Lin, Shieh-Yueh Yang, Herng-Er Horng, Che-Chuan Yang, Jen-Jie Chieh, Hsin-Hsien Chen, Bing-Hsien Liu, Ming-Jang Chiu
Objective: Parkinson's disease (PD) has significant clinical overlaps with atypical parkinsonism syndromes (APS), which have a poorer treatment response and a more aggressive course than PD. We aimed to identify plasma biomarkers to differentiate PD from APS. Methods: Plasma samples ( n = 204) were obtained from healthy controls and from patients with PD, dementia with Lewy bodies (DLB), multiple system atrophy, progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or frontotemporal dementia (FTD) with parkinsonism (FTD-P) or without parkinsonism...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29749079/acute-tau-knockdown-in-the-hippocampus-of-adult-mice-causes-learning-and-memory-deficits
#14
Ramon Velazquez, Eric Ferreira, An Tran, Emily C Turner, Ramona Belfiore, Caterina Branca, Salvatore Oddo
Misfolded and hyperphosphorylated tau accumulates in several neurodegenerative disorders including Alzheimer's disease, frontotemporal dementia with Parkinsonism, corticobasal degeneration, progressive supranuclear palsy, Down syndrome, and Pick's disease. Tau is a microtubule-binding protein, and its role in microtubule stabilization is well defined. In contrast, while growing evidence suggests that tau is also involved in synaptic physiology, a complete assessment of tau function in the adult brain has been hampered by robust developmental compensation of other microtubule-binding proteins in tau knockout mice...
May 10, 2018: Aging Cell
https://www.readbyqxmd.com/read/29698545/the-role-of-molecular-imaging-in-assessing-degenerative-parkinsonism-an-updated-review
#15
Nicolas Nicastro, Valentina Garibotto, Pierre R Burkhard
Diagnosing degenerative forms of parkinsonism still relies on a thorough clinical assessment, which in Parkinson's disease involves the presence of an asymmetric bradykinesia with rest tremor and/or rigidity that respond substantially to levodopa. Conversely, atypical forms, including multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration, exhibit additional features (cerebellar or pyramidal signs, early postural instability), a poor response to dopamine replacement therapy and a bad prognosis...
April 26, 2018: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29682240/extending-the-range-of-differential-diagnosis-of-chronic-traumatic-encephalopathy-of-the-boxer-insights-from-a-case-report
#16
Leonardo Caixeta, Iron Dangoni, Rafael Dias de Sousa, Pedro Paulo Dias Soares, Andreia Costa Rabelo Mendonça
Sports activities associated with repetitive cranial trauma have become a fad and are popular in gyms and even among children. It is important to consistently characterize the consequences of such sports activities in order to better advise society on the real risks to the central nervous system. We present the case of a former boxer reporting cognitive and behavioral symptoms that began six years after his retirement as a boxer, evolving progressively with parkinsonian and cerebellar features suggestive of probable chronic traumatic encephalopathy (CTE)...
January 2018: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29622555/mri-planimetry-and-magnetic-resonance-parkinsonism-index-in-the-differential-diagnosis-of-patients-with-parkinsonism
#17
V C Constantinides, G P Paraskevas, G Velonakis, P Toulas, E Stamboulis, E Kapaki
BACKGROUND AND PURPOSE: Differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration from Parkinson disease on clinical grounds is often difficult. MR imaging biomarkers could assist in a more accurate diagnosis. We examined the utility of MR imaging surface measurements (MR imaging planimetry) in the differential diagnosis of patients with parkinsonism. MATERIALS AND METHODS: Fifty-two patients with Parkinson-plus (progressive supranuclear palsy, n = 24; corticobasal degeneration, n = 9; multiple system atrophy, n = 19), 18 patients with Parkinson disease, and 15 healthy controls were included...
April 5, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29514947/clinical-value-of-neurofilament-and-phospho-tau-tau-ratio-in-the-frontotemporal-dementia-spectrum
#18
Lieke H H Meeter, Everard G Vijverberg, Marta Del Campo, Annemieke J M Rozemuller, Laura Donker Kaat, Frank Jan de Jong, Wiesje M van der Flier, Charlotte E Teunissen, John C van Swieten, Yolande A L Pijnenburg
OBJECTIVE: To examine the clinical value of neurofilament light chain (NfL) and the phospho-tau/total tau ratio (p/t-tau) across the entire frontotemporal dementia (FTD) spectrum in a large, well-defined cohort. METHODS: CSF NfL and p/t-tau levels were studied in 361 patients with FTD: 179 behavioral variant FTD, 17 FTD with motor neuron disease (FTD-MND), 36 semantic variant primary progressive aphasia (PPA), 19 nonfluent variant PPA, 4 logopenic variant PPA (lvPPA), 42 corticobasal syndrome, and 64 progressive supranuclear palsy...
April 3, 2018: Neurology
https://www.readbyqxmd.com/read/29496136/minimal-neuropathologic-diagnosis-for-brain-banking-in-the-normal-middle-aged-and-aged-brain-and-in-neurodegenerative-disorders
#19
Irina Alafuzoff
Research on human brain diseases is currently often conducted on cell cultures and animals. Several questions however can only be addressed by studying human postmortem brain tissue. However, brain tissue obtained postmortem almost always displays pathology that is often related to the aging phenomenon. Thus, in order to be certain that the answers obtained are reliable, a systematic and thorough assessment of the brain tissue to be studied should be carried out. We are currently aware of several protein alterations that are found in middle-aged and aged brains that are obtained from neurologically unimpaired subjects...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29453245/early-vs-late-age-at-onset-frontotemporal-dementia-and-frontotemporal-lobar-degeneration
#20
Sang Won Seo, Marie-Pierre Thibodeau, David C Perry, Alice Hua, Manu Sidhu, Isabel Sible, Jose Norberto S Vargas, Stephanie E Gaus, Gil D Rabinovici, Katherine D Rankin, Adam L Boxer, Joel H Kramer, Howard J Rosen, Maria Luisa Gorno-Tempini, Lea T Grinberg, Eric J Huang, Stephen J DeArmond, John Q Trojanowski, Bruce L Miller, William W Seeley
OBJECTIVE: To examine clinicopathologic correlations in early vs late age at onset frontotemporal dementia (FTD) and frontotemporal lobar degeneration (FTLD). METHODS: All patients were clinically evaluated and prospectively diagnosed at the UCSF Memory and Aging Center. Two consecutive series were included: (1) patients with a clinically diagnosed FTD syndrome who underwent autopsy (cohort 1) and (2) patients with a primary pathologic diagnosis of FTLD, regardless of the clinical syndrome (cohort 2)...
March 20, 2018: Neurology
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