keyword
MENU ▼
Read by QxMD icon Read
search

Corticobasal syndrome

keyword
https://www.readbyqxmd.com/read/27871202/corticobasal-syndrome-due-to-sporadic-creutzfeldt-jakob-disease-a-review-and-neuropsychological-case-report
#1
David Andrés González, Jason R Soble
OBJECTIVE: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disease with neuropsychological sequelae. This study highlighted a rare presentation of CJD (e.g. corticobasal syndrome [CBS]), reviewed updated diagnostic criteria and procedures for CJD (e.g. diffusion weighted imaging [DWI], real-time quaking-induced conversion [RT-QuIC]), and discussed differential diagnoses. METHOD: Case report methodology focused on a 68-year-old, Hispanic, right-handed man with 11 years of education...
November 22, 2016: Clinical Neuropsychologist
https://www.readbyqxmd.com/read/27814992/a-cluster-of-progranulin-c157kfsx97-mutations-in-southern-italy-clinical-characterization-and-genetic-correlations
#2
Cinzia Coppola, Dario Saracino, Gianfranco Puoti, Giacomo Lus, Clemente Dato, Isabelle Le Ber, Jeremie Pariente, Paola Caroppo, Elena Piccoli, Fabrizio Tagliavini, Giuseppe Di Iorio, Giacomina Rossi
Frontotemporal lobar degeneration (FTLD) is a group of neurodegenerative diseases displaying high clinical, pathologic, and genetic heterogeneity. Several autosomal dominant progranulin (GRN) mutations have been reported, accounting for 5%-10% of FTLD cases worldwide. In this study, we described the clinical characteristics of 7 Italian patients, 5 with a diagnosis of frontotemporal dementia behavioral variant and 2 of corticobasal syndrome (CBS), carrying the GRN deletion g.101349_101355delCTGCTGT, resulting in the C157KfsX97 null mutation, and hypothesized the existence of a founder effect by means of haplotype sharing analysis...
October 11, 2016: Neurobiology of Aging
https://www.readbyqxmd.com/read/27803826/a-corticobasal-syndrome-variant-of-familial-creutzfeldt-jakob-disease-with-stroke-like-onset
#3
Ján Necpál, Martin Stelzer, Silvia Koščová, Michal Patarák
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27802220/brain-regions-involved-in-arousal-and-reward-processing-are-associated-with-apathy-in-alzheimer-s-disease-and-frontotemporal-dementia
#4
Edward D Huey, Seonjoo Lee, Gayathri Cheran, Jordan Grafman, Davangere P Devanand
BACKGROUND: Apathy is a common and problematic symptom of several neurodegenerative illnesses, but its neuroanatomical bases are not understood. OBJECTIVE: To determine the regions associated with apathy in subjects with mild Alzheimer's disease (AD) using a method that accounts for the significant co-linearity of regional atrophy and neuropsychiatric symptoms. METHODS: We identified 57 subjects with mild AD (CDR = 1) and neuropsychiatric symptoms in the Alzheimer's Disease Neuroimaging Initiative (ADNI) database...
November 19, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27794115/in-vivo-visualization-of-tau-deposits-in-corticobasal-syndrome-by-18f-thk5351-pet
#5
Akio Kikuchi, Nobuyuki Okamura, Takafumi Hasegawa, Ryuichi Harada, Shoichi Watanuki, Yoshihito Funaki, Kotaro Hiraoka, Toru Baba, Naoto Sugeno, Ryuji Oshima, Shun Yoshida, Junpei Kobayashi, Michinori Ezura, Michiko Kobayashi, Ohito Tano, Shunji Mugikura, Ren Iwata, Aiko Ishiki, Katsutoshi Furukawa, Hiroyuki Arai, Shozo Furumoto, Manabu Tashiro, Kazuhiko Yanai, Yukitsuka Kudo, Atsushi Takeda, Masashi Aoki
OBJECTIVE: To determine whether (18)F-THK5351 PET can be used to visualize tau deposits in brain lesions in live patients with corticobasal syndrome (CBS). METHODS: We evaluated the in vitro binding of (3)H-THK5351 in postmortem brain tissues from a patient with corticobasal degeneration (CBD). In clinical PET studies, (18)F-THK5351 retention in 5 patients with CBS was compared to that in 8 age-matched normal controls and 8 patients with Alzheimer disease (AD). RESULTS: (3)H-THK5351 was able to bind to tau deposits in the postmortem brain with CBD...
November 29, 2016: Neurology
https://www.readbyqxmd.com/read/27792009/role-of-niemann-pick-type-c-disease-mutations-in-dementia
#6
Chiara Cupidi, Francesca Frangipane, Maura Gallo, Alessandra Clodomiro, Rosanna Colao, Livia Bernardi, Maria Anfossi, Maria Elena Conidi, Franca Vasso, Sabrina Anna Maria Curcio, Maria Mirabelli, Nicoletta Smirne, Giusi Torchia, Maria Gabriella Muraca, Gianfranco Puccio, Raffaele Di Lorenzo, Stefania Zampieri, Milena Romanello, Andrea Dardis, Raffaele Giovanni Maletta, Amalia Cecilia Bruni
BACKGROUND: Several neurological and systemic diseases can cause dementia, beyond Alzheimer's disease. Rare genetic causes are often responsible for dementia with atypical features. Recently, mutations causative for Niemann-Pick type C disease (NPC) have also been implicated in neurodegenerative diseases. NPC is an autosomal recessive lipid storage disorder caused by mutations in NPC1 and NPC2 genes. In adults, clinical presentation mimicking other neurodegenerative diseases makes diagnosis difficult...
October 24, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27774334/brain-mr-contribution-to-the-differential-diagnosis-of-parkinsonian-syndromes-an-update
#7
Giovanni Rizzo, Stefano Zanigni, Roberto De Blasi, Daniela Grasso, Davide Martino, Rodolfo Savica, Giancarlo Logroscino
Brain magnetic resonance (MR) represents a useful and feasible tool for the differential diagnosis of Parkinson's disease. Conventional MR may reveal secondary forms of parkinsonism and may show peculiar brain alterations of atypical parkinsonian syndromes. Furthermore, advanced MR techniques, such as morphometric-volumetric analyses, diffusion-weighted imaging, diffusion tensor imaging, tractography, proton MR spectroscopy, and iron-content sensitive imaging, have been used to obtain quantitative parameters useful to increase the diagnostic accuracy...
2016: Parkinson's Disease
https://www.readbyqxmd.com/read/27743520/characterizing-familial-corticobasal-syndrome-due-to-alzheimer-s-pathology-and-psen1-mutations
#8
Benjamin Lam, Aun Khan, Julia Keith, Ekaterina Rogaeva, Juan Bilbao, Peter St George-Hyslop, Mahdi Ghani, Morris Freedman, Donald T Stuss, Tiffany Chow, Sandra E Black, Mario Masellis
INTRODUCTION: Corticobasal syndrome (CBS) resulting from genetic Alzheimer's disease (AD) has been described only once. Whether familial CBS-AD is a distinct clinical entity with its own imaging signature remains unknown. METHODS: Four individuals with CBS from two families underwent detailed assessment. For two individuals, regional atrophy and hypoperfusion were compared to autopsy-confirmed typical late-onset AD and corticobasal degeneration, as well as genetically proven PSEN1 cases with an amnestic presentation...
October 12, 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/27742814/progression-of-brain-atrophy-in-psp-and-cbs-over-6-months-and-1-year
#9
Shubir Dutt, Richard J Binney, Hilary W Heuer, Phi Luong, Suneth Attygalle, Priyanka Bhatt, Gabe A Marx, Jonathan Elofson, Maria C Tartaglia, Irene Litvan, Scott M McGinnis, Bradford C Dickerson, John Kornak, Dana Waltzman, Lisa Voltarelli, Norbert Schuff, Gil D Rabinovici, Joel H Kramer, Clifford R Jack, Bruce L Miller, Howard J Rosen, Adam L Boxer
OBJECTIVE: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents. METHODS: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study...
November 8, 2016: Neurology
https://www.readbyqxmd.com/read/27714552/mapping-temporo-parietal-and-temporo-occipital-cortico-cortical-connections-of-the-human-middle-longitudinal-fascicle-in-subject-specific-probabilistic-and-stereotaxic-talairach-spaces
#10
Nikos Makris, A Zhu, G M Papadimitriou, P Mouradian, I Ng, E Scaccianoce, G Baselli, F Baglio, M E Shenton, Y Rathi, B Dickerson, E Yeterian, M Kubicki
Originally, the middle longitudinal fascicle (MdLF) was defined as a long association fiber tract connecting the superior temporal gyrus and temporal pole with the angular gyrus. More recently its description has been expanded to include all long postrolandic cortico-cortical association connections of the superior temporal gyrus and dorsal temporal pole with the parietal and occipital lobes. Despite its location and size, which makes MdLF one of the most prominent cerebral association fiber tracts, its discovery in humans is recent...
October 6, 2016: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/27662315/the-role-of-single-subject-brain-metabolic-patterns-in-the-early-differential-diagnosis-of-primary-progressive-aphasias-and-in-prediction-of-progression-to-dementia
#11
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
November 1, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27643900/parkinsonism
#12
Adrienne M Keener, Yvette M Bordelon
Parkinsonism is a clinical syndrome, which is characterized by bradykinesia, rigidity, rest tremor, and postural instability. Idiopathic Parkinson disease (PD) is the most common cause of this syndrome, though there are several other important etiologies that must be considered. These include the atypical Parkinsonian disorders multiple system atrophy (MSA), dementia with Lewy Bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS); as well as secondary causes of parkinsonism. These various disease entities may be distinguished based on key clinical features, which is critical for the purposes of diagnosis, treatment, and prognosis...
August 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27572945/cerebral-glucose-metabolism-and-dopaminergic-function-in-patients-with-corticobasal-syndrome
#13
Erik Mille, Johannes Levin, Matthias Brendel, Christian Zach, Henryk Barthel, Osama Sabri, Kai Bötzel, Peter Bartenstein, Adrian Danek, Axel Rominger
BACKGROUND AND PURPOSE: The corticobasal syndrome (CBS) is a clinical diagnosis that comprises a group of rare neurodegenerative diseases manifesting in movement disorder and cognitive impairment. While diagnosis is based upon clinical criteria, there have been a number of molecular imaging studies, albeit in rather small cohorts. Therefore, we investigated the pattern of cerebral glucose metabolism, as well as dopamine transporter (DAT) availability in a large and clinically well-defined cohort...
August 30, 2016: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/27561438/progressive-supranuclear-palsy-and-corticobasal-degeneration-diagnostic-challenges-and-clinicopathological-considerations
#14
A Eusebio, L Koric, O Félician, E Guedj, M Ceccaldi, J-P Azulay
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are two atypical parkinsonian syndromes first described half a century ago. The spectrum of these conditions as well as, more generally, the concept of tauopathy have dramatically changed over the past decade and especially in recent years. In particular, clinicopathological correlations have led to the description of several subtypes of these diseases and the features they share with other neurodegenerative diseases. The present paper is a review of how the concepts of PSP and CBD have evolved over time...
August 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27553583/visual-signs-and-symptoms-of-corticobasal-degeneration
#15
REVIEW
Richard A Armstrong
Corticobasal degeneration is a rare, progressive neurodegenerative disease and a member of the 'parkinsonian' group of disorders, which also includes Parkinson's disease, progressive supranuclear palsy, dementia with Lewy bodies and multiple system atrophy. The most common initial symptom is limb clumsiness, usually affecting one side of the body, with or without accompanying rigidity or tremor. Subsequently, the disease affects gait and there is a slow progression to influence ipsilateral arms and legs. Apraxia and dementia are the most common cortical signs...
November 2016: Clinical & Experimental Optometry: Journal of the Australian Optometrical Association
https://www.readbyqxmd.com/read/27543644/multimodal-imaging-evidence-of-pathology-mediated-disease-distribution-in-corticobasal-syndrome
#16
Corey T McMillan, Clara Boyd, Rachel G Gross, Jessica Weinstein, Kim Firn, Jon B Toledo, Katya Rascovsky, Leslie Shaw, David A Wolk, David J Irwin, Edward B Lee, John Q Trojanowski, Murray Grossman
OBJECTIVE: To use multimodal neuroimaging to evaluate the influence of heterogeneous underlying pathology in corticobasal syndrome (CBS) on the neuroanatomical distribution of disease. METHODS: We performed a retrospective evaluation of 35 patients with CBS with T1-weighted MRI, diffusion tensor imaging, and neuropathologic, genetic, or CSF evidence of underlying pathology. Patients were assigned to 2 groups: those with evidence of Alzheimer pathology (CBS-AD) and those without Alzheimer pathology (CBS-non-AD)...
September 20, 2016: Neurology
https://www.readbyqxmd.com/read/27529406/ftdp-17-with-pick-body-like-inclusions-associated-with-a-novel-tau-mutation-p-e372g
#17
Pawel Tacik, Michael A DeTure, Yari Carlomagno, Wen-Lang Lin, Melissa E Murray, Matthew C Baker, Keith A Josephs, Bradley F Boeve, Zbigniew K Wszolek, Neill R Graff-Radford, Joseph E Parisi, Leonard Petrucelli, Rosa Rademakers, Richard S Isaacson, Kenneth M Heilman, Ronald C Petersen, Dennis W Dickson, Naomi Kouri
Mutations in microtubule-associated protein tau gene (MAPT) cause frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). Here, we describe a patient with FTDP-17 and a novel missense mutation in exon 13 of MAPT, p.E372G. We compare clinicopathologic features of this patient to two previously unreported patients with another exon 13 mutation, p.G389R. The patient with the p.E372G mutation was a 40-year-old man with behavioral variant frontotemporal dementia (bvFTD), who subsequently developed agrammatic speech and parkinsonism...
October 5, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27495201/diagnostic-approach-to-atypical-parkinsonian-syndromes
#18
Nikolaus R McFarland
PURPOSE OF REVIEW: Although increasingly recognized, atypical parkinsonian syndromes remain challenging to diagnose and are underrecognized due to overlap with other parkinsonisms. This article provides a diagnostic approach to atypical parkinsonian syndromes, including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and dementia with Lewy bodies. The goal of this review is to aid the clinician in recognizing key clinical and pathologic features and to raise awareness of recent advances in diagnostics and treatment...
August 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27495200/treatment-of-advanced-parkinson-disease-and-related-disorders
#19
Janis M Miyasaki
PURPOSE OF REVIEW: Parkinson disease often spans decades of a patient's lifetime. Over time, nonmotor symptoms predominate and may limit dopaminergic therapy. Neurologists continue to play a vital role in treatment. In addition to balancing neurobehavioral complications of Parkinson disease with motor benefit, addressing nonmotor symptoms common in the advanced stage may improve quality of life and reduce symptom burden. Symptoms such as dysphagia, constipation, urinary dysfunction, orthostatic hypotension, and pain respond to nonpharmacologic and pharmacologic therapies...
August 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27489878/untangling-tau-imaging
#20
REVIEW
Victor L Villemagne, Nobuyuki Okamura, Christopher C Rowe
In vivo imaging of tau deposits is providing a better understanding of the temporal and spatial tau deposition in the brain, allowing a more comprehensive insight into the causes, diagnoses, and potentially treatment of tauopathies such as Alzheimer's disease, progressive supranuclear palsy, corticobasal syndrome, chronic traumatic encephalopathy, and some variants of frontotemporal lobar degeneration. The assessment of tau deposition in the brain over time will allow a deeper understanding of the relationship between tau and other variables such as cognition, genotype, and neurodegeneration, as well as assessing the role tau plays in ageing...
2016: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
keyword
keyword
113873
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"