1,043 results match your criteria Cortical Basal Ganglionic Degeneration


Cell-Type-Specific Decrease of the Intrinsic Excitability of Motor Cortical Pyramidal Neurons in Parkinsonism.

J Neurosci 2021 May 13. Epub 2021 May 13.

Department of Neurodegenerative Science, Van Andel Institute, 333 Bostwick Ave, N.E., Grand Rapids, MI 49503

The hypokinetic motor symptoms of Parkinson's disease (PD) are closely linked with a decreased motor cortical output as a consequence of elevated basal ganglia inhibition. However, whether and how the loss of dopamine alters the cellular properties of motor cortical neurons in PD remains undefined. We induced parkinsonism in adult C57BL6 mice of both sexes by injecting neurotoxin, 6-hydroxydopamine, into the medial forebrain bundle. Read More

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Structural and functional magnetic resonance imaging in isolated REM sleep behavior disorder: A systematic review of studies using neuroimaging software.

Sleep Med Rev 2021 Apr 22;59:101495. Epub 2021 Apr 22.

Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain; Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED:CB06/05/0018-ISCIII), Barcelona, Spain; Sleep Disorders Center, Neurology Service, Hospital Clínic, Barcelona, Catalonia, Spain.

Isolated rapid eye movement sleep behavior disorder (iRBD) is a harbinger for developing clinical synucleinopathies. Magnetic resonance imaging (MRI) has been suggested as a tool for understanding the brain bases of iRBD and its evolution. This review systematically analyzed original full text articles on structural and functional MRI in patients with video-polysomnography-confirmed iRBD according to systematic procedures suggested by Reviews and Meta-analyses (PRISMA). Read More

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Synaptic Zinc: An Emerging Player in Parkinson's Disease.

Int J Mol Sci 2021 Apr 29;22(9). Epub 2021 Apr 29.

CNRS, Laboratoire de Neurosciences Cognitives, (UMR 729), Aix Marseille Université, 13331 Marseille, France.

Alterations of zinc homeostasis have long been implicated in Parkinson's disease (PD). Zinc plays a complex role as both deficiency and excess of intracellular zinc levels have been incriminated in the pathophysiology of the disease. Besides its role in multiple cellular functions, Zn also acts as a synaptic transmitter in the brain. Read More

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A longitudinal observation of brain structure between AD and FTLD.

Clin Neurol Neurosurg 2021 Mar 24;205:106604. Epub 2021 Mar 24.

Department of Neurology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address:

Introduction: Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) are the leading causes of dementia. To better understand the disease development of cognitive function and anatomical structure in AD and FTLD, we analyzed the changes in brain volume by MRI and the psychological test results. Here, we report a dynamic observation of brain structure. Read More

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An autopsy-proven case of Corticobasal degeneration heralded by Pontine infarction.

BMC Neurol 2021 Apr 6;21(1):148. Epub 2021 Apr 6.

Department of Neurology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, 23 Kyungheedae-ro, Dongdaemun-gu, Seoul, 02447, Republic of Korea.

Background: Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Read More

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Crossed cerebellar diaschisis on F-FDG PET: Frequency across neurodegenerative syndromes and association with C-PIB and F-Flortaucipir.

J Cereb Blood Flow Metab 2021 Mar 10:271678X211001216. Epub 2021 Mar 10.

Memory and Aging Center, Department of Neurology, Weill Institute for Neurosciences, University of California San Francisco, San Francisco, CA, USA.

We used F-FDG-PET to investigate the frequency of crossed cerebellar diaschisis (CCD) in 197 patients with various syndromes associated with neurodegenerative diseases. In a subset of 117 patients, we studied relationships between CCD and cortical asymmetry of Alzheimer's pathology (β-amyloid (C-PIB) and tau (F-Flortaucipir)). PET images were processed using MRIs to derive parametric SUVR images and define regions of interest. Read More

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Cerebrospinal Fluid Proteins Altered in Corticobasal Degeneration.

Mov Disord 2021 05 4;36(5):1278-1280. Epub 2021 Mar 4.

Department of Clinical Neuroscience, Neuro Svenningsson, J5:20 Bioclinicum, Karolinska Universitetssjukhuset, Stockholm, Sweden.

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18F-AV-1451 positron emission tomography in neuropathological substrates of corticobasal syndrome.

Brain 2021 02;144(1):266-277

Department of Neurology, Massachusetts General Hospital, Charlestown, MA, USA.

Multiple neuropathological processes can manifest in life as a corticobasal syndrome. We sought to relate retention of the tau-PET tracer 18F-AV-1451 and structural magnetic resonance measures of regional atrophy to clinical features in clinically diagnosed and neuropathologically confirmed cases of corticobasal syndrome and to determine whether these vary with the underlying neuropathological changes. In this observational, cross-sectional study, 11 subjects (eight female and three male, median age 72 years) with corticobasal syndrome underwent structural MRI, tau-PET with 18F-AV-1451, amyloid-PET with 11C-Pittsburgh compound B, detailed clinical examinations and neuropsychological testing. Read More

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February 2021

Grey matter volume abnormalities in the first depressive episode of medication-naïve adult individuals: a systematic review of voxel based morphometric studies.

Int J Psychiatry Clin Pract 2020 Dec 22:1-14. Epub 2020 Dec 22.

Departments of Psychiatry, College of Medicine, Korea University, Seoul, South Korea.

Background: To identify the reliable and consistent grey matter volume (GMV) abnormalities associated with major depressive disorder (MDD), we excluded the influence of confounding clinical characteristics, comorbidities and brain degeneration on brain morphological abnormalities by inclusion of non-comorbid and non-geriatric drug-naïve MDD individuals experiencing first episode depressive.

Methods: The PubMed, Scopus, Web of Science, Science Direct and Google scholar databases were searched for papers published in English up to April 2020.

Results: A total of 21 voxel based morphometric (VBM) studies comparing 845 individuals in the first depressive episode and medication-naïve with 940 healthy control subjects were included. Read More

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December 2020

Synaptic Dysfunction in Huntington's Disease: Lessons from Genetic Animal Models.

Neuroscientist 2020 Nov 16:1073858420972662. Epub 2020 Nov 16.

IDDRC, Jane and Terry Semel Institute for Neuroscience & Human Behavior, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.

The understanding of the functional and structural changes occurring in the cerebral cortex and basal ganglia in Huntington's disease (HD) has benefited considerably from the generation of genetic animal models. Most studies of synaptic alterations in HD models have focused on the striatum, but a more complete picture of synaptic dysfunction in the cortico-basal ganglia-cortical loop is emerging. Here, we provide a review and analysis of current developments in the study of synaptic alterations in these areas using HD rodent models. Read More

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November 2020

Energy Metabolism Decline in the Aging Brain-Pathogenesis of Neurodegenerative Disorders.

Metabolites 2020 Nov 7;10(11). Epub 2020 Nov 7.

Department of Human Motor Behavior, Jerzy Kukuczka Academy of Physical Education, 40-065 Katowice, Poland.

There is a growing body of evidencethat indicates that the aging of the brain results from the decline of energy metabolism. In particular, the neuronal metabolism of glucose declines steadily, resulting in a growing deficit of adenosine triphosphate (ATP) production-which, in turn, limits glucose access. This vicious circle of energy metabolism at the cellular level is evoked by a rising deficiency of nicotinamide adenine dinucleotide (NAD) in the mitochondrial salvage pathway and subsequent impairment of the Krebs cycle. Read More

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November 2020

Subthalamic beta oscillations correlate with dopaminergic degeneration in experimental parkinsonism.

Exp Neurol 2021 01 24;335:113513. Epub 2020 Oct 24.

Movement Disorders and Neuromodulation Unit, Department of Neurology, Charité University Medicine Berlin, Berlin, Germany; German Center for Neurodegenerative Diseases (DZNE), Germany; Berlin Institute of Health (BIH), Berlin, Germany; Department of Neurology, University Medicine Göttingen, Göttingen, Germany. Electronic address:

Excessive beta activity has been shown in local field potential recordings from the cortico-basal ganglia loop of Parkinson's disease patients and in its various animal models. Recent evidence suggests that enhanced beta oscillations may play a central role in the pathophysiology of the disorder and that beta activity may be directly linked to the motor impairment. However, the temporal evolution of exaggerated beta oscillations during the ongoing dopaminergic neurodegeneration and its relation to the motor impairment and histological changes are still unknown. Read More

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January 2021

The Cortical Basal ganglia Functional Scale (CBFS): Development and preliminary validation.

Parkinsonism Relat Disord 2020 Aug 25;79:121-126. Epub 2020 Aug 25.

Department of Neurology, Memory and Aging Center, University of California, San Francisco, CA, USA.

Objective: To develop a patient/care-giver reported scale capable of easily and reliably assessing functional disability in 4 repeat tauopathies (4RTs).

Background: 4R tauopathies including progressive supranuclear palsy, corticobasal degeneration and a subset of frontotemporal dementias manifest a range of overlapping clinical phenotypes. No available rating scale is capable of evaluating the functional impact of these complex disorders. Read More

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Sensitivity-Specificity of Tau and Amyloid β Positron Emission Tomography in Frontotemporal Lobar Degeneration.

Ann Neurol 2020 11 12;88(5):1009-1022. Epub 2020 Sep 12.

Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.

Objective: To examine associations between tau and amyloid β (Aβ) molecular positron emission tomography (PET) and both Alzheimer-related pathology and 4-repeat tau pathology in autopsy-confirmed frontotemporal lobar degeneration (FTLD).

Methods: Twenty-four patients had [ F]-flortaucipir-PET and died with FTLD (progressive supranuclear palsy [PSP], n = 10; corticobasal degeneration [CBD], n = 10; FTLD-TDP, n = 3; and Pick disease, n = 1). All but 1 had Pittsburgh compound B (PiB)-PET. Read More

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November 2020

Anatomical and neurochemical bases of theory of mind in de novo Parkinson's Disease.

Cortex 2020 09 10;130:401-412. Epub 2020 Jul 10.

Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy; IRCCS Ospedale Policlinico S. Martino, Genoa, Italy. Electronic address:

Theory of mind (ToM) deficit is a frequent finding in subjects with neurological and psychiatric conditions. While a number of brain regions play a role in ToM, to date the contribution of the diffuse projection systems is less understood. Here, we explored the topographical and neurochemical bases of ToM using multi-tracer molecular imaging and quantitative electroencephalography (qEEG) in a group of 30 drug-naïve, de novo Parkinson's Disease (PD) patients (mean age 73. Read More

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September 2020

Individual Brain Metabolic Signatures in Corticobasal Syndrome.

J Alzheimers Dis 2020 ;76(2):517-528

Nuclear Medicine Unit, San Raffaele Hospital, Milan, Italy.

Background: Corticobasal syndrome (CBS) is the usual clinical presentation of patients with corticobasal degeneration pathology. Nevertheless, there are CBS individuals with postmortem neuropathology typical of Alzheimer's disease (AD).

Objective: In this study, we aim to detect FDG-PET metabolic signatures at the single-subject level in a CBS sample, also evaluated with cerebrospinal fluid (CSF) markers for AD pathology. Read More

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Neurobiologic Rationale for Treatment of Apathy in Alzheimer's Disease With Methylphenidate.

Am J Geriatr Psychiatry 2021 01 5;29(1):51-62. Epub 2020 May 5.

Johns Hopkins University School of Medicine (NJ, PBR), Baltimore, MD.

The public health burden of Alzheimer's disease (AD) is related not only to cognitive symptoms, but also to neuropsychiatric symptoms, including apathy. Apathy is defined as a quantitative reduction of goal-directed activity in comparison to a previous level of functioning and affects 30%-70% of persons with AD. Previous attempts to treat apathy in AD-both nonpharmacologically and pharmacologically-have been wanting. Read More

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January 2021

Spinocerebellar Ataxia Type 3: A Case Report and Literature Review.

J Neuropathol Exp Neurol 2020 06;79(6):641-646

Department of Pathology.

Spinocerebellar ataxia type 3 (SCA3), also known by the eponym Machado-Joseph disease, is an autosomal dominant CAG trinucleotide (polyglutamine) repeat disease that presents in young- to middle-aged adults. SCA3 was first described in Azorean individuals and has interesting epidemiological patterns. It is characterized clinically by progressive ataxia and neuropathologically by progressive degenerative changes in the spinal cord and cerebellum, along with degeneration of the cortex and basal ganglia. Read More

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Proactive interference apraxic agraphia: a writing and drawing disorder associated with corticobasal syndrome.

Neurocase 2020 06 23;26(3):125-130. Epub 2020 Apr 23.

Division of Cognitive and Behavioral Neurology, Department of Neurology, University of Florida College of Medicine , Gainesville, USA.

Proactive interference is when a previously performed task impairs performance on a current task. It is often associated with memory tasks and has not been reported to interfere with writing or drawing. We evaluated a left-handed man diagnosed with corticobasal syndrome who had a two-year history of progressive agraphia. Read More

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Corticobasal Degeneration Reveals Its Signature by Tau Strains.

Mov Disord 2020 06 7;35(6):934. Epub 2020 Apr 7.

Center for Neurodegenerative Diseases, Department of Medicine, Surgery and Dentistry, University of Salerno, Salerno, Italy.

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Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3.

Front Neurol 2020 3;11:124. Epub 2020 Mar 3.

Brain Research Center, National Yang-Ming University, Taipei, Taiwan.

Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss of the cerebellar cortex and degeneration of the cerebral cortex. We used the three-dimensional fractal dimension (3D-FD) method to quantify the morphological changes in the supratentorial regions and assessed atrophy in the relatively focal regions in patients with SCA3. Read More

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A probable role of copper in the comorbidity in Wilson's and Creutzfeldt-Jakob's Diseases: a case report.

Virol J 2020 03 13;17(1):35. Epub 2020 Mar 13.

Aristotle University, 1st Neurology clinic, AHEPA Hospital, Thessaloniki, Greece.

Background: To the best of our knowledgedd, there is currently no case in the literature reporting the comorbidity of Wilson's and Creutzfeldt-Jakob disease (CJD), linked through copper.

Case Presentation: A 44-year-old male with a history of inherited Wilson's disease (hepatolenticular degeneration), which manifested as mild liver injury and psychiatric symptoms, was admitted to our department due to speech and cognitive disturbances. Upon his admission, he had motor aphasia as well as psychomotor retardation with an otherwise normal neurological examination. Read More

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Novel tau filament fold in corticobasal degeneration.

Nature 2020 04 12;580(7802):283-287. Epub 2020 Feb 12.

MRC Laboratory of Molecular Biology, Cambridge, UK.

Corticobasal degeneration (CBD) is a neurodegenerative tauopathy-a class of disorders in which the tau protein forms insoluble inclusions in the brain-that is characterized by motor and cognitive disturbances. The H1 haplotype of MAPT (the tau gene) is present in cases of CBD at a higher frequency than in controls, and genome-wide association studies have identified additional risk factors. By histology, astrocytic plaques are diagnostic of CBD; by SDS-PAGE, so too are detergent-insoluble, 37 kDa fragments of tau. Read More

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Thalamostriatal degeneration contributes to dystonia and cholinergic interneuron dysfunction in a mouse model of Huntington's disease.

Acta Neuropathol Commun 2020 02 7;8(1):14. Epub 2020 Feb 7.

Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 University street, H3A 2B4, Montreal, Quebec, Canada.

Huntington's disease (HD) is an autosomal dominant trinucleotide repeat disorder characterized by choreiform movements, dystonia and striatal neuronal loss. Amongst multiple cellular processes, abnormal neurotransmitter signalling and decreased trophic support from glutamatergic cortical afferents are major mechanisms underlying striatal degeneration. Recent work suggests that the thalamostriatal (TS) system, another major source of glutamatergic input, is abnormal in HD although its phenotypical significance is unknown. Read More

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February 2020

Primary Tau Pathology, Not Copathology, Correlates With Clinical Symptoms in PSP and CBD.

J Neuropathol Exp Neurol 2020 03;79(3):296-304

From the Penn Alzheimer's Disease Core Center.

Distinct neuronal and glial tau pathologies define corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Additional Alzheimer disease, TDP-43, and Lewy body copathologies are also common. The interplay of these pathologies with clinical symptoms remains unclear as individuals can present with corticobasal syndrome, frontotemporal dementia, PSP, or atypical Parkinsonism and may have additional secondary impairments. Read More

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Fulminant corticobasal degeneration: a distinct variant with predominant neuronal tau aggregates.

Acta Neuropathol 2020 04 16;139(4):717-734. Epub 2020 Jan 16.

Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, University College London, 1 Wakefield Street, London, WC1N 1PJ, UK.

Corticobasal degeneration typically progresses gradually over 5-7 years from onset till death. Fulminant corticobasal degeneration cases with a rapidly progressive course were rarely reported (RP-CBD). This study aimed to investigate their neuropathological characteristics. Read More

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Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy.

Neuropathology 2020 Jun 10;40(3):287-294. Epub 2020 Jan 10.

Department of Neurology, Tokyo Teishin Hospital, Tokyo, Japan.

Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66-year-old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened. Read More

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Brain and Body: A Review of Central Nervous System Contributions to Movement Impairments in Diabetes.

Diabetes 2020 01;69(1):3-11

Department of Physical Therapy, Faculty of Medicine, University of British Columbia, Vancouver, Canada

Diabetes is associated with a loss of somatosensory and motor function, leading to impairments in gait, balance, and manual dexterity. Data-driven neuroimaging studies frequently report a negative impact of diabetes on sensorimotor regions in the brain; however, relationships with sensorimotor behavior are rarely considered. The goal of this review is to consider existing diabetes neuroimaging evidence through the lens of sensorimotor neuroscience. Read More

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January 2020

Disentangling brain functional network remodeling in corticobasal syndrome - A multimodal MRI study.

Neuroimage Clin 2020 2;25:102112. Epub 2019 Dec 2.

Max Planck Institute for Human Cognitive and Brain Sciences Leipzig, Germany; Clinic for Cognitive Neurology, University Clinic, Leipzig, Germany.

Objective: The clinical diagnosis of corticobasal syndrome (CBS) represents a challenge for physicians and reliable diagnostic imaging biomarkers would support the diagnostic work-up. We aimed to investigate the neural signatures of CBS using multimodal T1-weighted and resting-state functional magnetic resonance imaging (MRI).

Methods: Nineteen patients with CBS (age 67. Read More

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January 2021

Widespread subcortical grey matter degeneration in primary lateral sclerosis: a multimodal imaging study with genetic profiling.

Neuroimage Clin 2019 12;24:102089. Epub 2019 Nov 12.

Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, 152-160 Pearse Street, Dublin 2, Ireland. Electronic address:

Background: Primary lateral sclerosis (PLS) is a low incidence motor neuron disease which carries a markedly better prognosis than amyotrophic lateral sclerosis (ALS). Despite sporadic reports of extra-motor symptoms, PLS is widely regarded as a pure upper motor neuron disorder. The post mortem literature of PLS is strikingly sparse and very little is known of subcortical grey matter pathology in this condition. Read More

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September 2020