2,318 results match your criteria: "Cortical Basal Ganglionic Degeneration"
J Neurol
December 2024
Department of Neurology and Alzheimer Centre, Erasmus MC University Medical Centre (Erasmus MC), Dr. Molenwaterplein 40, 3015 CE, Rotterdam, The Netherlands.
Background: Frontotemporal lobar degeneration (FTLD) is one of the leading causes of early onset dementia. Pathogenic variants in GRN have been reported to cause 5-25% of familial and 5% of sporadic FTLD. Here, we present two novel, likely pathogenic variants in GRN.
View Article and Find Full Text PDFAlzheimer's disease (AD) is a progressive neurodegenerative disorder, characterized by the presence of extracellular amyloid plaques consisting of β-amyloid peptides (Aβ) and intracellular neurofibrillary tangles (NFTs) composed of hyperphosphorylated tau (pTau) protein in the brain. Genetic and animal studies strongly indicate that Aβ, tau and neuroinflammation play important roles in the pathogenesis of AD. Several staging models showed that NFTs correlated well with the disease progression.
View Article and Find Full Text PDFIdeggyogy Sz
November 2024
Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, UK.
Corticobasal degeneration (CBD) is one of the primary tauopathies with a disease onset in the 5th to 7th decade. CBD is a progressive condition of unknown aetiology, which is characterised neuropathologically by neuronal loss, astrogliosis and deposition of filamentous tau inclusions, composed entirely of 4-repeat tau isoforms, in neurons and glial cells in cerebral cortical areas, basal ganglia, brainstem and cerebellar nuclei. The term CBD is now a neuropathological diagnostic one and for the canonical clinical syndrome associated with CBD neuropathological changes, the corticobasal syndrome (CBS) term is used.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
November 2024
Department of Neurology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Stavrou Niarchou Av., University Campus, Ioannina, Greece.
Mol Neurodegener
November 2024
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Int J Mol Sci
November 2024
Laboratory of Pathoneurochemistry, Department of Neurochemistry, Mossakowski Medical Research Institute, 02-106 Warsaw, Poland.
Neurology
December 2024
From the Department of Neurology, Vanderbilt University Medical Center, Nashville, TN.
Background And Objectives: Frontotemporal lobar degeneration (FTLD) includes different clinical syndromes with distinct patterns of symptoms and neuroanatomical locations of neurodegeneration. However, FTLD is clinically heterogeneous (with overlapping symptoms across several domains) and neuroanatomically heterogeneous (with brain atrophy in different locations in different patients). Traditional methods struggle to fully account for this heterogeneity.
View Article and Find Full Text PDFClin Park Relat Disord
October 2024
Department of Molecular Genetics, Brain Research Institute, Niigata University, 1-757 Asahimachi-dori, Chuoku, Niigata 951-8585, Japan.
Introduction: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) manifest with variable clinical features. We initiated a multicenter prospective registry study-the Japanese Longitudinal Biomarker Study in PSP and CBD-in November 2014 at 45 Japanese institutions to collect clinical information and biological samples to elucidate the natural courses and diagnostic biomarkers of PSP/CBD.
Methods: Initial symptoms, clinical features, and scores (Progressive Supranuclear Palsy Rating Scale [PSPRS], Barthel Index, Mini-Mental State Examination, and Frontal Assessment Battery) of patients clinically diagnosed with PSP/corticobasal syndrome (CBS) at the first registration were analyzed.
Neurology
December 2024
From the Department of Neuroscience (H.S., M.K., S.K., A.M., A.R.M., D.W.D.), and Department of Neurology (P.W.T., R.J.U., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL.
J Biol Chem
November 2024
Institute of Molecular Embryology and Genetics (IMEG), Department of Genomic Neurology, Kumamoto University, Kumamoto, Japan; Graduate School of Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan. Electronic address:
Tau aggregation is a defining feature of neurodegenerative tauopathies, including Alzheimer's disease, corticobasal degeneration, and frontotemporal dementia. This aggregation involves the liquid-liquid phase separation (LLPS) of Tau, followed by its sol-gel phase transition, representing a crucial step in aggregate formation both in vitro and in vivo. However, the precise cofactors influencing Tau phase transition and aggregation under physiological conditions (e.
View Article and Find Full Text PDFNat Chem Biol
October 2024
Department of Medical Genetics, University of Cambridge, Cambridge Institute for Medical Research, Cambridge, UK.
bioRxiv
October 2024
Center for Alzheimer's and Neurodegenerative Diseases, Peter O'Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center, Dallas, TX.
Neurodegenerative tauopathies are characterized by the deposition of distinct fibrillar tau assemblies whose rigid core structures correlate with defined neuropathological phenotypes. Essential tremor (ET) is a progressive neurological disease that, in some cases, is associated with cognitive impairment and tau accumulation. Consequently, we explored the tau assembly conformation in ET patients with tau pathology using cytometry-based tau biosensor assays.
View Article and Find Full Text PDFBiomedicines
October 2024
Department of Neurology, Medical University of Warsaw, Kondratowicza 8, 03-242 Warsaw, Poland.
J Clin Med
September 2024
Department of Neurology, Medical University of Warsaw, 03-242 Warsaw, Poland.
Atypical parkinsonian syndromes (APSs) are a group of neurodegenerative disorders that differ from idiopathic Parkinson's disease (IPD) in their clinical presentation, underlying pathology, and response to treatment. APSs include conditions such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and dementia with Lewy bodies (DLB). These disorders are characterized by a combination of parkinsonian features and additional symptoms, such as autonomic dysfunction, supranuclear gaze palsy, and asymmetric motor symptoms.
View Article and Find Full Text PDFJ Neurol
December 2024
Cerebral Function Unit, Manchester Centre for Clinical Neurosciences, Northern Care AllianceNHS Foundation Trust, Salford, UK.
Neurobiol Dis
October 2024
Department of Neurology, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan; Department of Diagnosis, Prevention and Treatment of Dementia, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan; Department of Research for Parkinson's Disease, Juntendo University Graduate School of Medicine, Tokyo 113-8421, Japan; Neurodegenerative Disorders Collaborative Laboratory, RIKEN Center for Brain Science, 2-1 Hirosawa, Wako-shi, Saitama 351-0198, Japan. Electronic address:
Corticobasal degeneration (CBD) is a major four-repeat tauopathy along with progressive supranuclear palsy (PSP). Although detergent-insoluble 37-40-kDa carboxyl-terminal tau fragments (CTFs) are hallmarks of CBD pathology, the process of their formation is unknown. This study monitored the formation of CBD-type fibrils that exhibit astrocytic plaques, a characteristic CBD pathology, using its biochemical properties different from those of Alzheimer's disease/PSP-type fibrils.
View Article and Find Full Text PDFBrain Pathol
October 2024
Brain and Mind Centre and School of Medical Sciences, University of Sydney, Camperdown, New South Wales, Australia.
Amyotroph Lateral Scler Frontotemporal Degener
September 2024
Computational Neuroimaging Group, School of Medicine, Trinity College Dublin, Dublin, Ireland and.
Alzheimers Dement
November 2024
Rainwater Charitable Foundation, Fort Worth, Texas, USA.
The tauopathies are defined by pathological tau protein aggregates within a spectrum of clinically heterogeneous neurodegenerative diseases. The primary tauopathies meet the definition of rare diseases in the United States. There is no approved treatment for primary tauopathies.
View Article and Find Full Text PDFeNeurologicalSci
December 2024
Department of Neuropathology (Brain Bank for Aging Research), Tokyo Metropolitan Institute for Geriatrics and Gerontology, Tokyo, Japan.
A 72-year-old man presented with a 6-month history of decreased voluntary speech. Sparse speech and decreased word fluency were observed. Articulation, naming, comprehension, and repetition were preserved.
View Article and Find Full Text PDFCurr Med Res Opin
October 2024
1st Department of Neurology, Aiginition University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Introduction: Corticobasal syndrome (CBS) is a rare form of atypical parkinsonism, most commonly caused by neurodegenerative disorders. Autoimmune underlying conditions are extremely rare, and anti-Yo antibody-associated CBS has not been reported yet.
Case Report: Herein, we describe a case of a 68-year-old woman presenting with progressive dysarthria, gait instability and difficulty using her left hand with subacute deterioration during the last 3 months.
Mov Disord
December 2024
Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Background: Seed amplification assay (SAA) testing has been developed as a biomarker for the diagnosis of α-synuclein-related neurodegenerative disorders.
Objective: The objective of this study was to assess the rate of α-synuclein SAA positivity in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) and to analyze clinical and pathological features of SAA-positive and -negative cases.
Methods: A total of 96 cerebrospinal fluid samples from clinically diagnosed PSP (n = 59) and CBS (n = 37) cases were analyzed using α-synuclein SAA.
Psychogeriatrics
November 2024
Department of Psychiatry, School of Medicine, University of Occupational and Environmental Health, Fukuoka, Japan.
NPJ Parkinsons Dis
September 2024
Pharmacology Unit, Department of Pathology and Experimental Therapeutics, School of Medicine and Health Sciences, Institute of Neurosciences, University of Barcelona, 08907, L'Hospitalet de Llobregat, Spain.
The orphan G protein-coupled receptor 37 (GPR37), widely associated with Parkinson's disease (PD), undergoes proteolytic processing under physiological conditions. The N-terminus domain is proteolyzed by a disintegrin and metalloproteinase 10 (ADAM-10), which generates various membrane receptor forms and ectodomain shedding (ecto-GPR37) in the extracellular environment. We investigated the processing and density of GPR37 in several neurodegenerative conditions, including Lewy body disease (LBD), multiple system atrophy (MSA), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), and Alzheimer's disease (AD).
View Article and Find Full Text PDFBiochem J
September 2024
Division of Neuroscience, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester M13 9PT, U.K.