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Corticobasal syndrome

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https://www.readbyqxmd.com/read/28510312/the-midbrain-to-pons-ratio-distinguishes-progressive-supranuclear-palsy-from-non-fluent-primary-progressive-aphasias
#1
M Silsby, R Y Tweedie-Cullen, C R Murray, G M Halliday, J R Hodges, J R Burrell
BACKGROUND AND PURPOSE: To determine the clinical utility of the midbrain-to-pons (M/P) ratio as a clinical biomarker of progressive supranuclear palsy (PSP) in patients with non-fluent primary progressive aphasia syndromes. METHODS: Patients with PSP, progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA) were recruited. Patients were diagnosed clinically, but pathological confirmation was available in a proportion of patients. Midbrain and pons areas were measured using Osirix Lite, a free DICOM viewer...
May 16, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28507319/longitudinal-changes-of-tau-pet-imaging-in-relation-to-hypometabolism-in-prodromal-and-alzheimer-s-disease-dementia
#2
K Chiotis, L Saint-Aubert, E Rodriguez-Vieitez, A Leuzy, O Almkvist, I Savitcheva, M Jonasson, M Lubberink, A Wall, G Antoni, A Nordberg
The development of tau-specific positron emission tomography (PET) tracers allows imaging in vivo the regional load of tau pathology in Alzheimer's disease (AD) and other tauopathies. Eighteen patients with baseline investigations enroled in a 17-month follow-up study, including 16 with AD (10 had mild cognitive impairment and a positive amyloid PET scan, that is, prodromal AD, and six had AD dementia) and two with corticobasal syndrome. All patients underwent PET scans with [(18)F]THK5317 (tau deposition) and [(18)F]FDG (glucose metabolism) at baseline and follow-up, neuropsychological assessment at baseline and follow-up and a scan with [(11)C]PIB (amyloid-β deposition) at baseline only...
May 16, 2017: Molecular Psychiatry
https://www.readbyqxmd.com/read/28500752/which-ante-mortem-clinical-features-predict-progressive-supranuclear-palsy-pathology
#3
Gesine Respondek, Carolin Kurz, Thomas Arzberger, Yaroslau Compta, Elisabet Englund, Leslie W Ferguson, Ellen Gelpi, Armin Giese, David J Irwin, Wassilios G Meissner, Christer Nilsson, Alexander Pantelyat, Alex Rajput, John C van Swieten, Claire Troakes, Keith A Josephs, Anthony E Lang, Brit Mollenhauer, Ulrich Müller, Jennifer L Whitwell, Angelo Antonini, Kailash P Bhatia, Yvette Bordelon, Jean-Christophe Corvol, Carlo Colosimo, Richard Dodel, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw Morris, Peter Nestor, Wolfgang H Oertel, Gil D Rabinovici, James B Rowe, Thilo van Eimeren, Gregor K Wenning, Adam Boxer, Lawrence I Golbe, Irene Litvan, Maria Stamelou, Günter U Höglinger
BACKGROUND: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. OBJECTIVE: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. METHODS: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology...
May 13, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28486594/apathy-and-impulsivity-in-frontotemporal-lobar-degeneration-syndromes
#4
Claire J Lansdall, Ian T S Coyle-Gilchrist, P Simon Jones, Patricia Vázquez Rodríguez, Alicia Wilcox, Eileen Wehmann, Katrina M Dick, Trevor W Robbins, James B Rowe
Apathy and impulsivity are common and disabling consequences of frontotemporal lobar degeneration. They cause substantial carer distress, but their aetiology remains elusive. There are critical limitations to previous studies in this area including (i) the assessment of either apathy or impulsivity alone, despite their frequent co-existence; (ii) the assessment of behavioural changes within single diagnostic groups; and (iii) the use of limited sets of tasks or questions that relate to just one aspect of these multifactorial constructs...
May 9, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28484383/when-playing-is-a-problem-an-atypical-case-of-alien-hand-syndrome-in-a-professional-pianist
#5
Arantxa Alfaro, Ángela Bernabeu, Francisco J Badesa, Nicolas García, Eduardo Fernández
Alien hand syndrome (AHS) is a neurological illness characterized by limb movements which are carried out without being aware of it. Many patients describe these movements as aggressive and some perceive a strong feeling of estrangement and go so far as to deny ownership. The sense of body ownership is the perception that parts of one's body pertain to oneself, despite it is moving or not and if movement is intentional or unintentional. These anomalous self-experiences may arise in patients with focal brain lesions and provide unique opportunities to disclose the neural components underlying self-body perception...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28474385/disrupted-topological-organization-of-structural-networks-revealed-by-probabilistic-diffusion-tractography-in-tourette-syndrome-children
#6
Hongwei Wen, Yue Liu, Islem Rekik, Shengpei Wang, Jishui Zhang, Yue Zhang, Yun Peng, Huiguang He
Tourette syndrome (TS) is a childhood-onset neurobehavioral disorder. Although previous TS studies revealed structural abnormalities in distinct corticobasal ganglia circuits, the topological alterations of the whole-brain white matter (WM) structural networks remain poorly understood. Here, we used diffusion MRI probabilistic tractography and graph theoretical analysis to investigate the topological organization of WM networks in 44 drug-naive TS children and 41 age- and gender-matched healthy children. The WM networks were constructed by estimating inter-regional connectivity probability and the topological properties were characterized using graph theory...
May 5, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28469972/a-wolf-in-sheep-s-clothing-an-alien-leg-in-corticobasal-syndrome
#7
Diana Angelika Olszewska, Allan McCarthy, Brian Murray, Brian Magennis, Sean Connolly, Tim Lynch
BACKGROUND: Alien limb phenomenon occurs in 50-60% of patients with corticobasal syndrome (CBS) and usually presents with an "alien hand" phenomenon. The "alien foot" presentation is rarer and may be misdiagnosed, as foot involvement can lead to erroneous localization of the clinical problem to the knee, hip, or back. Subsequently misdiagnoses such as myelopathy, radiculopathy, functional disorder, stiff leg syndrome, neuromyotonia, and painful leg moving toes syndrome may occur. CASE REPORT: We describe two patients with alien foot symptoms that resulted in multiple opinions from different specialists, multiple diagnostic and therapeutic procedures, and delayed diagnosis...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28455846/tool-use-in-neurodegenerative-diseases-planning-or-technical-reasoning
#8
Josselin Baumard, Mathieu Lesourd, Chrystelle Remigereau, Christophe Jarry, Frédérique Etcharry-Bouyx, Valérie Chauviré, François Osiurak, Didier Le Gall
Recent works showed that tool use can be impaired in stroke patients because of either planning or technical reasoning deficits, but these two hypotheses have not yet been compared in the field of neurodegenerative diseases. The aim of this study was to address the relationships between real tool use, mechanical problem-solving, and planning skills in patients with Alzheimer's disease (AD, n = 32), semantic dementia (SD, n = 16), and corticobasal syndrome (CBS, n = 9). Patients were asked to select and use ten common tools, to solve three mechanical problems, and to complete the Tower of London test...
April 29, 2017: Journal of Neuropsychology
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#9
REVIEW
Nicholas T Olney, Salvatore Spina, Bruce L Miller
Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome...
May 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/28390475/corrigendum-to-gray-and-white-matter-structural-changes-in-corticobasal-syndrome-neurobiol-aging-37-2016-82-90
#10
Neeraj Upadhyay, Antonio Suppa, Maria Cristina Piattella, Flavio Di Stasio, Nikolaos Petsas, Claudio Colonnese, Carlo Colosimo, Alfredo Berardelli, Patrizia Pantano
No abstract text is available yet for this article.
May 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28389938/combined-visual-and-semi-quantitative-assessment-of-123-i-fp-cit-spect-for-the-diagnosis-of-dopaminergic-neurodegenerative-diseases
#11
Jun Ueda, Hajime Yoshimura, Keiji Shimizu, Megumu Hino, Nobuo Kohara
Visual and semi-quantitative assessments of (123)I-FP-CIT single-photon emission computed tomography (SPECT) are useful for the diagnosis of dopaminergic neurodegenerative diseases (dNDD), including Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. However, the diagnostic value of combined visual and semi-quantitative assessment in dNDD remains unclear. Among 239 consecutive patients with a newly diagnosed possible parkinsonian syndrome who underwent (123)I-FP-CIT SPECT in our medical center, 114 patients with a disease duration less than 7 years were diagnosed as dNDD with the established criteria or as non-dNDD according to clinical judgment...
April 7, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28386688/beyond-the-midbrain-atrophy-wide-spectrum-of-structural-mri-finding-in-cases-of-pathologically-proven-progressive-supranuclear-palsy
#12
REVIEW
Keita Sakurai, Aya M Tokumaru, Keigo Shimoji, Shigeo Murayama, Kazutomi Kanemaru, Satoru Morimoto, Ikuko Aiba, Motoo Nakagawa, Yoshiyuki Ozawa, Masashi Shimohira, Noriyuki Matsukawa, Yoshio Hashizume, Yuta Shibamoto
PURPOSE: Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. METHODS: With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated...
May 2017: Neuroradiology
https://www.readbyqxmd.com/read/28378615/the-utility-of-fdg-pet-in-the-differential-diagnosis-of-parkinsonism
#13
Leposava Brajkovic, Vladimir Kostic, Dragana Sobic-Saranovic, Elka Stefanova, Milica Jecmenica-Lukic, Ana Jesic, Milica Stojiljkovic, Strahinja Odalovic, Francesca Gallivanone, Isabella Castiglioni, Branislava Radovic, Goran Trajkovic, Vera Artiko
INTRODUCTION: Differential diagnosis of parkinsonian disorders can be difficult on clinical grounds, especially in the early stage. Recent advancements in 18-F-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging reveals different patterns of regional glucose metabolism in idiopathic Parkinson's disease (IPD) and atypical parkinsonian syndromes, such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), which may help differentiating between these conditions...
April 5, 2017: Neurological Research
https://www.readbyqxmd.com/read/28360997/imaging-biomarkers-in-parkinson-s-disease-and-parkinsonian-syndromes-current-and-emerging-concepts
#14
REVIEW
Usman Saeed, Jordana Compagnone, Richard I Aviv, Antonio P Strafella, Sandra E Black, Anthony E Lang, Mario Masellis
Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson's disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson's disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous disorders that may share some clinical features with Parkinson's disease, but are uncommon distinct clinicopathological diseases...
2017: Translational Neurodegeneration
https://www.readbyqxmd.com/read/28322905/bilingualism-delays-the-onset-of-behavioral-but-not-aphasic-forms-of-frontotemporal-dementia
#15
Suvarna Alladi, Thomas H Bak, Mekala Shailaja, Divyaraj Gollahalli, Amulya Rajan, Bapiraju Surampudi, Michael Hornberger, Vasanta Duggirala, Jaydip Ray Chaudhuri, Subhash Kaul
Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently...
March 18, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28318985/functional-disconnection-of-thalamic-and-cerebellar-dentate-nucleus-networks-in-progressive-supranuclear-palsy-and-corticobasal-syndrome
#16
Neeraj Upadhyay, Antonio Suppa, Maria Cristina Piattella, Costanza Giannì, Matteo Bologna, Flavio Di Stasio, Nikolaos Petsas, Francesca Tona, Giovanni Fabbrini, Alfredo Berardelli, Patrizia Pantano
AIM: To assess functional rearrangement following neurodegeneration in the thalamus and dentate nucleus in patients with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). METHODS: We recruited 19 patients with PSP, 11 with CBS and 14 healthy subjects. All the subjects underwent resting-state (rs) fMRI using a 3T system. Whole brain functional connectivity of the thalamus and dentate nucleus were calculated by means of a seed-based approach with FEAT script in FSL toolbox...
March 15, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28318282/altered-resting-state-eeg-complexity-in-children-with-tourette-syndrome-a-preliminary-study
#17
Wen-Chin Weng, Chi-Feng Chang, Lee Chin Wong, Jui-Hsiang Lin, Wang-Tso Lee, Jiann-Shing Shieh
OBJECTIVE: Tourette syndrome is a developmental neuropsychiatric disorder in children, and abnormal corticobasal ganglion connectivity is implied for the pathophysiology. Multiscale entropy, an entropy-based method to measure dynamic complexity at multiple temporal scales, is helpful to disclose the information of brain connectivity. This preliminary study investigated the complexity of resting-state electroencephalogram signals using multiscale entropy in children with Tourette syndrome...
March 20, 2017: Neuropsychology
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
#18
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...
May 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28259709/consensus-classification-of-posterior-cortical-atrophy
#19
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/28243754/differentiation-of-atypical-parkinson-syndromes
#20
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
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