1,018 results match your criteria Cortical Basal Ganglionic Degeneration


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

Am J Geriatr Psychiatry 2020 May 5. 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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jagp.2020.04.026DOI Listing

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

J Neuropathol Exp Neurol 2020 Jun;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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/jnen/nlaa033DOI Listing

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fneur.2020.00124DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7062793PMC

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12985-020-01309-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7071643PMC

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41586-020-2043-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7148158PMC

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s40478-020-0878-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7007676PMC
February 2020

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2337/db19-0321DOI Listing
January 2020

Corticobasal degeneration.

Int Rev Neurobiol 2019 21;149:87-136. Epub 2019 Nov 21.

Edmond J. Safra Program in Parkinson's Disease and the Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, Toronto, Ontario, Canada; Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

Corticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by the predominance of pathological 4 repeat tau deposition in various cell types and anatomical regions. Corticobasal syndrome (CBS) is one of the clinical phenotypes associated with CBD pathology, manifesting as a progressive asymmetric akinetic-rigid, poorly levodopa-responsive parkinsonism, with cerebral cortical dysfunction. CBD can manifest as several clinical phenotypes, and similarly, CBS can also have a pathologic diagnosis other than CBD. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/bs.irn.2019.10.014DOI Listing

[Clinical and neuroimaging features in 6 patients with corticobasal syndrome].

Zhonghua Nei Ke Za Zhi 2019 Dec;58(12):905-907

Department of Neurology, Perking University Third Hospital, Beijing 100191, China.

The clinical and imaging data in 6 patients with corticobasal syndrome were retrospectively analyzed. Six patients presented asymmetric clinical symptoms, including 5 with cognitive impairment, 6 with emotional disorders, 2 with cortical sensory deficit, 5 with lalopathy, and 4 with apraxia. All patients developed limb dystonia and limb or trunk stiffness, 4 with tumble, 4 with bradykinesia, and 2 with tremor. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.0578-1426.2019.12.007DOI Listing
December 2019

Overexpression of Sirtuin 1 protein in neurons prevents and reverses experimental diabetic neuropathy.

Brain 2019 12;142(12):3737-3752

Department of Neurology, University of Maryland School of Medicine, Baltimore, MD 21201, USA.

In diabetic neuropathy, there is activation of axonal and sensory neuronal degeneration pathways leading to distal axonopathy. The nicotinamide-adenine dinucleotide (NAD+)-dependent deacetylase enzyme, Sirtuin 1 (SIRT1), can prevent activation of these pathways and promote axonal regeneration. In this study, we tested whether increased expression of SIRT1 protein in sensory neurons prevents and reverses experimental diabetic neuropathy induced by a high fat diet (HFD). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/brain/awz324DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6885680PMC
December 2019

Dynamic Changes in Brain Glucose Metabolism and Neuronal Structure in Rats with Heart Failure.

Neuroscience 2020 01 6;424:34-44. Epub 2019 Nov 6.

Key Laboratory of Chinese Internal Medicine of Ministry of Education and Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing 100700, China. Electronic address:

Patients with heart failure (HF) are more susceptible to cognitive impairment, but the mechanism is still unclear. This study aimed to observe the dynamic changes in brain glucose metabolism and neuronal structure in different stages of HF. An HF rat model was established by ligating the anterior descending branch of the left coronary artery. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuroscience.2019.10.008DOI Listing
January 2020

F-AV1451 PET imaging and multimodal MRI changes in progressive supranuclear palsy.

J Neurol 2020 Feb 22;267(2):341-349. Epub 2019 Oct 22.

Department of Clinical Neurosciences, University of Cambridge, Herchel Smith Building, Forvie Site, Robinson Way, Cambridge Biomedical Campus, Cambridge, CB2 0SZ, UK.

Objectives: Progressive supranuclear palsy (PSP) is characterized by deposition of straight filament tau aggregates in the grey matter (GM) of deep nuclei and cerebellum. We examined the relationship between tau pathology (assessed via F-AV1451 PET) and multimodal MRI imaging using GM volume, cortical thickness (CTh), and diffusion tensor imaging (DTI).

Methods: Twenty-three people with clinically probable PSP-Richardson's syndrome (age 68. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00415-019-09566-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6989441PMC
February 2020

Cerebrovascular pathology presenting as corticobasal syndrome: An autopsy case series of "vascular CBS".

Parkinsonism Relat Disord 2019 11 2;68:79-84. Epub 2019 Sep 2.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road S, Jacksonville, FL, 32224, USA. Electronic address:

Background: The corticobasal syndrome (CBS) is heterogeneous in terms of postmortem neuropathology. While it has been previously studied with antemortem neuroimaging, clinicopathologic features of corticobasal syndrome associated with cerebrovascular pathology (vascular CBS) have yet to be reported.

Methods: To identify vascular CBS, we searched the database of the CurePSP Brain Bank for patients with a clinical diagnosis of CBS who failed to meet neuropathologic criteria for corticobasal degeneration (CBD) or other neurodegenerative disease processes, but who had significant cerebrovascular pathology. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.parkreldis.2019.09.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141792PMC
November 2019

Therapeutic potential of cannabinoids as neuroprotective agents for damaged cells conducing to movement disorders.

Int Rev Neurobiol 2019 28;146:229-257. Epub 2019 Jun 28.

Department of Pharmacology, Faculty of Medicine and Nursing, University of the Basque Country (UPV/EHU), Leioa, Spain; Neurodegenerative Diseases Group, BioCruces Bizkaia Health Research Institute, Barakaldo, Bizkaia, Spain. Electronic address:

The basal ganglia (BG), an organized network of nuclei that integrates cortical information, play a crucial role in controlling motor function. In fact, movement disorders such as Parkinson's disease (PD) and Huntington's disease (HD) are caused by the degeneration of specific structures within the BG. There is substantial evidence supporting the idea that cannabinoids may constitute novel promising compounds for the treatment of movement disorders as neuroprotective and anti-inflammatory agents. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/bs.irn.2019.06.012DOI Listing
February 2020

Potential usefulness of signal intensity of cerebral gyri on quantitative susceptibility mapping for discriminating corticobasal degeneration from progressive supranuclear palsy and Parkinson's disease.

Neuroradiology 2019 Nov 2;61(11):1251-1259. Epub 2019 Jul 2.

Department of Radiology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, 807-8555, Japan.

Purpose: The typical MRI findings in corticobasal degeneration (CBD), which have been described in previous reports, may be non-specific. We evaluated cerebral gyri (CG) using quantitative susceptibility mapping (QSM) images of patients with CBD, progressive supranuclear palsy (PSP), and Parkinson's disease (PD) to determine the possibility of discriminating them on an individual basis.

Methods: After reviewing the normal appearances on QSM on 16 healthy subjects, two radiologists assessed abnormal findings from 12 CBD, 14 PSP, and 30 PD patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00234-019-02253-6DOI Listing
November 2019
5 Reads

Different Dopaminergic Dysfunctions Underlying Parkinsonian Akinesia and Tremor.

Front Neurosci 2019 29;13:550. Epub 2019 May 29.

National Research Council, Institute of Cognitive Sciences and Technologies, Rome, Italy.

Although the occurrence of Parkinsonian akinesia and tremor is traditionally associated to dopaminergic degeneration, the multifaceted neural processes that cause these impairments are not fully understood. As a consequence, current dopamine medications cannot be tailored to the specific dysfunctions of patients with the result that generic drug therapies produce different effects on akinesia and tremor. This article proposes a computational model focusing on the role of dopamine impairments in the occurrence of akinesia and resting tremor. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fnins.2019.00550DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6549580PMC
May 2019
4 Reads

Neuropathological correlates of structural and functional imaging biomarkers in 4-repeat tauopathies.

Brain 2019 07;142(7):2068-2081

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

Neurodegenerative dementia syndromes are characterized by spreading of pathological protein deposition along syndrome-specific neural networks. Structural and functional MRI measures can assess the integrity of these networks and have been proposed as biomarkers of disease progression for clinical trials. The relationship between in vivo imaging measures and pathological features, at the single subject level, remains largely unknown. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/brain/awz122DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598736PMC
July 2019
22 Reads

Aberrant Coupling Between Resting-State Cerebral Blood Flow and Functional Connectivity in Wilson's Disease.

Front Neural Circuits 2019 18;13:25. Epub 2019 Apr 18.

Laboratory of Digital Medical Imaging, Medical Imaging Center, The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei, China.

Both abnormalities of resting-state cerebral blood flow (CBF) and functional connectivity in Wilson's disease (WD) have been identified by several studies. Whether the coupling of CBF and functional connectivity is imbalanced in WD remains largely unknown. To assess this possibility, 27 patients with WD and 27 sex- and age-matched healthy controls were recruited to acquire functional MRI and arterial spin labeling imaging data. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fncir.2019.00025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6482267PMC
January 2020
4 Reads

Primary progressive dysarthria as the initial presentation of corticobasal degeneration: A case report.

Rev Neurol (Paris) 2019 May 1;175(5):336-338. Epub 2019 May 1.

Department of Neuroscience, Azienda Ospedaliera San Camillo Forlanini, C.ne Gianicolense 87, Rome, Italy.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neurol.2018.08.011DOI Listing
May 2019
1 Read

Rare histotype of sporadic Creutzfeldt-Jakob disease, clinically suspected as corticobasal degeneration.

BMJ Case Rep 2019 Mar 7;12(3). Epub 2019 Mar 7.

Institute of Neuroscience, Newcastle University, Newcastle Upon Tyne, UK.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disease that can mimic other neurological disorders. We present a case of sCJD in a 64-year-old man that presented with corticobasal syndrome and survived for 3 years. He presented initially with dementia, hemiparkinsonism and alien limb phenomenon and was diagnosed with corticobasal degeneration, ultimately progressing to immobility and akinetic mutism. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2018-228305DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6424260PMC
March 2019
4 Reads

Tau PET in autosomal dominant Alzheimer's disease: relationship with cognition, dementia and other biomarkers.

Brain 2019 04;142(4):1063-1076

Mallinckrodt Institute of Radiology, Washington University in St. Louis, MO, USA.

Tauopathy is a hallmark pathology of Alzheimer's disease with a strong relationship with cognitive impairment. As such, understanding tau may be a key to clinical interventions. In vivo tauopathy has been measured using cerebrospinal fluid assays, but these do not provide information about where pathology is in the brain. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/brain/awz019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439328PMC
April 2019
84 Reads
9.196 Impact Factor

Corticobasal syndrome: neuroimaging and neurophysiological advances.

Eur J Neurol 2019 May 15;26(5):701-e52. Epub 2019 Mar 15.

IRCCS Neuromed Institute, 'Sapienza' University of Rome, Pozzilli (Isernia), Italy.

Corticobasal degeneration (CBD) is a neurodegenerative condition characterized by 4R tau protein deposition in several brain regions that clinically manifests itself as a heterogeneous atypical parkinsonism typically expressed in adulthood. The prototypical clinical phenotype of CBD is corticobasal syndrome (CBS). Important insights into the pathophysiological mechanisms underlying motor and higher cortical symptoms in CBS have been gained by using advanced neuroimaging and neurophysiological techniques. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ene.13928DOI Listing
May 2019
7 Reads

Clinical, neuropsychological and imaging characteristics of Alzheimer's disease patients presenting as corticobasal syndrome.

J Neurol Sci 2019 Mar 29;398:142-147. Epub 2019 Jan 29.

1st Department of Neurology, National and Kapodistrian University of Athens, School of Medicine, Eginition Hospital, Athens, Greece, 72-74 Vas. Sophias Ave., 11528 Athens, Greece.

Background: Corticobasal syndrome (CBS) can harbor diverse pathologies, such as corticobasal degeneration (CBD) and Alzheimer's disease (AD). CSF biochemical analysis in CBS patients can confidently distinguish between an AD (CBS-AD) and a non-AD (CBS-nAD) pathology.

Objective: We utilized classical CSF biomarkers to make a distinction between the two groups and examine their clinical, neuropsychological, neuropsychiatric and imaging differences. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jns.2019.01.046DOI Listing
March 2019
2 Reads

Globular glial tauopathy Type II: Clinicopathological study of two autopsy cases.

Neuropathology 2019 Apr 15;39(2):111-119. Epub 2019 Jan 15.

Department of Pathology, Brain Research Institute, Niigata University, Niigata, Japan.

Globular glial tauopathies (GGTs) are four-repeat tauopathies characterized by the presence of two types of tau-positive globular glial inclusions (GGIs): globular oligodendrocytic and astrocytic inclusions (GOIs and GAIs). GGTs are classified into three different neuropathological subtypes: Types I, II and III. We report two patients with GGTs - a 76-year-old woman and a 70-year-old man - in whom the disease duration was 5 and 6 years, respectively. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/neup.12532DOI Listing
April 2019
13 Reads

Cerebellar degeneration correlates with motor symptoms in Huntington disease.

Ann Neurol 2019 03 4;85(3):396-405. Epub 2019 Feb 4.

Centre for Brain Research, University of Auckland, Auckland, New Zealand.

Objective: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder characterized by variable motor and behavioral symptoms attributed to major neuropathology of mainly the basal ganglia and cerebral cortex. The role of the cerebellum, a brain region involved in the coordination of movements, in HD neuropathology has been controversial. This study utilizes postmortem human brain tissue to investigate whether Purkinje cell degeneration in the neocerebellum is present in HD, and how this relates to disease symptom profiles. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ana.25413DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6590792PMC
March 2019
50 Reads

Loss-of-Huntingtin in Medial and Lateral Ganglionic Lineages Differentially Disrupts Regional Interneuron and Projection Neuron Subtypes and Promotes Huntington's Disease-Associated Behavioral, Cellular, and Pathological Hallmarks.

J Neurosci 2019 03 9;39(10):1892-1909. Epub 2019 Jan 9.

Roslyn and Leslie Goldstein Laboratory for Stem Cell Biology and Regenerative Medicine,

Emerging studies are providing compelling evidence that the pathogenesis of Huntington's disease (HD), a neurodegenerative disorder with frequent midlife onset, encompasses developmental components. Moreover, our previous studies using a hypomorphic model targeting huntingtin during the neurodevelopmental period indicated that loss-of-function mechanisms account for this pathogenic developmental component (Arteaga-Bracho et al., 2016). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1523/JNEUROSCI.2443-18.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6407290PMC
March 2019
14 Reads

Evaluating the cerebral correlates of survival in amyotrophic lateral sclerosis.

Ann Clin Transl Neurol 2018 Nov 23;5(11):1350-1361. Epub 2018 Sep 23.

Neuroscience and Mental Health Institute University of Alberta Edmonton Canada.

Objective: To evaluate cerebral degenerative changes in ALS and their correlates with survival using 3D texture analysis.

Methods: A total of 157 participants were included in this analysis from four neuroimaging studies. Voxel-wise texture analysis on T1-weighted brain magnetic resonance images (MRIs) was conducted between patients and controls. Read More

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1002/acn3.655
Publisher Site
http://dx.doi.org/10.1002/acn3.655DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6243384PMC
November 2018
16 Reads

PET Molecular Imaging in Atypical Parkinsonism.

Int Rev Neurobiol 2018 8;142:3-36. Epub 2018 Oct 8.

Newcastle University Newcastle Magnetic Resonance Centre & Positron Emission Tomography Centre, Newcastle University Campus for Ageing & Vitality, Newcastle upon Tyne, United Kingdom; Department of Clinical Medicine-Positron Emission Tomography Centre, Aarhus University, Aarhus, Denmark. Electronic address:

Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration are three neurodegenerative disorders characterized by parkinsonism along with involvement of other brain cortical and subcortical regions. The ante mortem diagnosis of these disorders is extremely challenging with up to a quarter of these patients being misdiagnosed, particularly in the early stages of disease. While highly specific and sensitive imaging biomarkers of individual atypical parkinsonisms have not been identified yet, molecular PET and SPECT imaging have improved our knowledge of the physiopathology and neuropathology of these disorders and are often used as supportive criteria for the differential diagnosis of these conditions. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00747742183010
Publisher Site
http://dx.doi.org/10.1016/bs.irn.2018.09.001DOI Listing
May 2019
16 Reads
1.921 Impact Factor

Regional subcortical shape analysis in premanifest Huntington's disease.

Hum Brain Mapp 2019 04 30;40(5):1419-1433. Epub 2018 Oct 30.

Center for Imaging Science, Johns Hopkins University, Baltimore, Maryland.

Huntington's disease (HD) involves preferential and progressive degeneration of striatum and other subcortical regions as well as regional cortical atrophy. It is caused by a CAG repeat expansion in the Huntingtin gene, and the longer the expansion the earlier the age of onset. Atrophy begins prior to manifest clinical signs and symptoms, and brain atrophy in premanifest expansion carriers can be studied. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/hbm.24456DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420821PMC
April 2019
36 Reads

Pathophysiology of corticobasal degeneration: Insights from neurophysiological studies.

J Clin Neurosci 2019 Feb 13;60:17-23. Epub 2018 Oct 13.

Department of Neurology, Christian Doppler Klinik, Paracelsus Medical University, Salzburg, Austria; Centre for Cognitive Neurosciences Salzburg, Salzburg, Austria; University for Medical Informatics and Health Technology, UMIT, Hall in Tirol, Austria.

Background: Several studies have applied electrophysiological techniques to physiologically characterize corticobasal degeneration (CBD).

Methods: We performed a systematic literature search of these studies and reviewed all 25 identified articles.

Results: Conventional electroencephalography (EEG) is usually normal even in the late stages of disease. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S09675868183082
Publisher Site
http://dx.doi.org/10.1016/j.jocn.2018.10.027DOI Listing
February 2019
39 Reads
1.320 Impact Factor

Imaging of Motor Cortex Physiology in Parkinson's Disease.

Mov Disord 2018 11 2;33(11):1688-1699. Epub 2018 Oct 2.

Department of Applied Physiology and Kinesiology, University of Florida, Gainesville, Florida, USA.

There is abundant evidence that the pathophysiology of Parkinson's disease (PD) is not confined to the nigrostriatal dopaminergic pathway but propagates along the cortico-basal ganglia-thalamo-cortical neural network. A critical node in this functional circuit impacted by PD is the primary motor cortex (M1), which plays a key role in generating neural impulses that regulate movements. The past several decades have lay witness to numerous in vivo neuroimaging techniques that provide a window into the function and structure of M1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mds.102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261674PMC
November 2018
4 Reads

A Cortical Pathogenic Theory of Parkinson's Disease.

Neuron 2018 09;99(6):1116-1128

CINAC, Hospital Universitario HM Puerta del Sur, Móstoles, Universidad CEU-San Pablo, Madrid, Spain; CIBERNED, Instituto de Salud Carlos III, Madrid, Spain. Electronic address:

In Parkinson's disease, the progressive neurodegeneration of nigrostriatal dopaminergic neurons in the substantia nigra pars compacta (SNc) is associated with classic motor features, which typically have a focal onset. Since a defined somatotopic arrangement in the SNc has not been recognized, this focal motor onset is unexplained and hardly justified by current pathogenic theories of bottom-up disease progression (Braak's hypothesis, prionopathy). Here we propose that corticostriatal activity may represent a critical somatotopic "stressor" for nigrostriatal terminals, ultimately driving retrograde nigrostriatal degeneration and leading to focal motor onset and progression of Parkinson's disease. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuron.2018.07.028DOI Listing
September 2018
6 Reads

Regenerating islet-derived 1α (REG-1α) protein increases tau phosphorylation in cell and animal models of tauopathies.

Neurobiol Dis 2018 11 6;119:136-148. Epub 2018 Aug 6.

MMDN, Univ. Montpellier, EPHE, INSERM, U1198, PSL University, Montpellier F-34095, France. Electronic address:

REG-1α, a secreted protein containing a C-type lectin domain, is expressed in various organs and plays different roles in digestive system cells in physiological and pathological conditions. Like other members of the Reg family, REG-1α is expressed also in the brain where it has different functions. For instance, we previously reported that REG-1α regulates neurite outgrowth and is overexpressed during the very early stages of Alzheimer's disease (AD). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2018.07.029DOI Listing
November 2018
13 Reads

Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease.

Neuroimage Clin 2018 19;20:236-242. Epub 2018 Feb 19.

Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Although much prior work has focused on the basal ganglia and cortical pathology that defines Huntington's disease (HD), recent studies have also begun to characterize cerebral white matter damage (Rosas et al., 2006; Dumas et al., 2012; Poudel et al. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nicl.2018.01.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078048PMC
January 2019
37 Reads

Bilateral upper limb rehabilitation with videogame-based feedback in corticobasal degeneration: a case reports study.

Neurocase 2018 06 17;24(3):156-160. Epub 2018 Jul 17.

b Clinical Laboratory of Experimental Neurorehabilitation , Santa Lucia Foundation, IRCCS , Rome , Italy.

Corticobasal degeneration (CBD) is a neurodegenerative disorder characterized by a combination of cortical and basal ganglia signs. We reported two cases treated with a bilateral upper limb rehabilitation tool with videogame based feedback for 3 time per week for 8 weeks. Both patients showed an improvement of pinch and grasp forces and motor function. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/13554794.2018.1499938DOI Listing
June 2018
10 Reads

In vivo quantification of glial activation in minipigs overexpressing human α-synuclein.

Synapse 2018 12 12;72(12):e22060. Epub 2018 Aug 12.

Department of Nuclear Medicine and PET Center, Institute of Clinical Medicine, Aarhus University and Hospital, Aarhus, Denmark.

Parkinson's disease is characterized by a progressive loss of substantia nigra (SN) dopaminergic neurons and the formation of Lewy bodies containing accumulated alpha-synuclein (α-syn). The pathology of Parkinson's disease is associated with neuroinflammatory microglial activation, which may contribute to the ongoing neurodegeneration. This study investigates the in vivo microglial and dopaminergic response to overexpression of α-syn. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/syn.22060DOI Listing
December 2018
17 Reads

mutations cause widespread white matter and basal ganglia abnormalities, but restricted cortical damage.

Neuroimage Clin 2018 9;19:848-857. Epub 2018 Jun 9.

Department of Neurology, University of Campinas (UNICAMP), Campinas, Brazil. Electronic address:

mutations are the major cause of autosomal recessive Hereditary Spastic Paraplegia. The disease has a wide phenotypic variability indicating many regions of the nervous system besides the corticospinal tract are affected. Despite this, anatomical and phenotypic characterization is restricted. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nicl.2018.05.031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6008284PMC
January 2019
36 Reads

Diffuse Lewy body disease manifesting as corticobasal syndrome: A rare form of Lewy body disease.

Neurology 2018 07 13;91(3):e268-e279. Epub 2018 Jun 13.

From the Departments of Neuroscience (K.K., M.E.M., S.K., N.S., J.A.V.G., D.W.D.), Psychiatry and Psychology (T.J.F., A.P.), and Neurology (T.K., R.J.U., N.R.G.-R., Z.K.W.), Mayo Clinic, Jacksonville, FL; and Behavioral Neurology (K.A.J.), Department of Neurology, Mayo Clinic, Rochester, MN.

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). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1212/WNL.0000000000005828DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6059030PMC
July 2018
24 Reads

A progressive breakdown of the body in space.

Neurocase 2018 06 8;24(3):133-139. Epub 2018 Jun 8.

a Neuropsychology and Language Disorders Unit, Department of Neurology , School of Medicine, National and Kapodistrian University of Athens , Greece.

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. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/13554794.2018.1482356DOI Listing
June 2018
22 Reads

In Vivo Multidimensional Brain Imaging in Huntington's Disease Animal Models.

Methods Mol Biol 2018 ;1780:285-301

CEA, DRF, Institut de biologie François Jacob, Molecular Imaging Research Center (MIRCen), Fontenay-aux-Roses, France.

Huntington's disease (HD) is a genetic neurodegenerative disorder caused by an abnormal expansion of a CAG repeat located in the gene encoding for huntingtin protein. This mutation induces the expression of a polyglutamine stretch in the mutated protein resulting in the modification of various biological properties of the wild-type protein and the progressive appearance of motor, cognitive, and psychiatric disorders that are typically associated to this condition. Although the exact neuropathological mechanisms of degeneration are still not fully understood, HD pathology is characterized by severe neuronal losses in various brain regions including the basal ganglia and many cortical areas. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/978-1-4939-7825-0_15DOI Listing
February 2019
48 Reads

Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration.

Neurocase 2018 04 25;24(2):111-120. Epub 2018 May 25.

a Department of Radiology , Mayo Clinic , Rochester , MN , USA.

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. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/13554794.2018.1477963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6095655PMC
April 2018
12 Reads

The patchy tremor landscape: recent advances in pathophysiology.

Curr Opin Neurol 2018 08;31(4):455-461

Donders Institute for Brain, Cognition and Behaviour, Centre for Cognitive Neuroimaging, Radboud University.

Purpose Of Review: We focus on new insights in the pathophysiology of Parkinson's disease tremor, essential tremor, tremor in dystonia, and orthostatic tremor.

Recent Findings: Neuroimaging findings suggest that Parkinson's disease resting tremor is associated with dopaminergic dysfunction, serotonergic dysfunction, or both. Not all tremors in Parkinson's disease have the same pathophysiology: postural tremor in Parkinson's disease can be subdivided into pure postural tremor, which involves nondopaminergic mechanisms, and re-emergent tremor, which has a dopaminergic basis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/WCO.0000000000000582DOI Listing
August 2018
6 Reads

Expression of CRYM in different rat organs during development and its decreased expression in degenerating pyramidal tracts in amyotrophic lateral sclerosis.

Neuropathology 2018 Jun 30;38(3):247-259. Epub 2018 Mar 30.

Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

The protein μ-crystallin (CRYM) is a novel component of the marsupial lens that has two functions: it is a key regulator of thyroid hormone transportation and a reductase of sulfur-containing cyclic ketimines. In this study, we examined changes of the expression pattern of CRYM in different rat organs during development using immunohistochemistry and immunoblotting. As CRYM is reportedly expressed in the corticospinal tract, we also investigated CRYM expression in human cases of amyotrophic lateral sclerosis (ALS) using immunohistochemistry. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/neup.12466DOI Listing
June 2018
14 Reads

Synaptic plasticity and levodopa-induced dyskinesia: electrophysiological and structural abnormalities.

J Neural Transm (Vienna) 2018 08 28;125(8):1263-1271. Epub 2018 Feb 28.

Laboratory of Neurophysiology, IRCCS Fondazione Santa Lucia c/o CERC, via del Fosso di Fiorano 64, 00143, Rome, Italy.

Parkinson's disease (PD) is a neurodegenerative disorder characterized by progressive degeneration of dopaminergic neurons located in the midbrain. The gold-standard therapy for PD is the restoration of dopamine (DA) levels through the chronic administration of the DA precursor levodopa (L-DOPA). Although levodopa therapy is the main therapeutic approach for PD, its use is limited by the development of very disabling dyskinetic movements, mainly due to the fluctuation of DA cerebral content. Read More

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s00702-018-1864-6
Publisher Site
http://dx.doi.org/10.1007/s00702-018-1864-6DOI Listing
August 2018
9 Reads

Connectivity-based characterisation of subcortical grey matter pathology in frontotemporal dementia and ALS: a multimodal neuroimaging study.

Brain Imaging Behav 2018 Dec;12(6):1696-1707

Quantitative Neuroimaging Group, Academic Unit of Neurology, Biomedical Sciences Institute, Trinity College Dublin, Pearse Street, Dublin, Ireland.

Frontotemporal dementia (FTD) phenotypes have distinctive and well-established cortical signatures, but their subcortical grey matter profiles are poorly characterised. The comprehensive characterisation of striatal and thalamic pathology along the ALS-FTD spectrum is particularly timely, as dysfunction of frontostriatal and cortico-thalamic networks contribute to phenotype-defining cognitive, behavioral, and motor deficits. Ten patients with behavioral-variant FTD, 11 patients with non-fluent-variant primary progressive aphasia, 5 patients with semantic-variant primary progressive aphasia, 14 ALS-FTD patients with C9orf72 hexanucleotide expansions, 12 ALS-FTD patients without hexanucleotide repeats, 36 ALS patients without cognitive impairment and 50 healthy controls were included in a prospective neuroimaging study. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s11682-018-9837-9DOI Listing
December 2018
37 Reads

The multisystem degeneration amyotrophic lateral sclerosis - neuropathological staging and clinical translation.

Arch Ital Biol 2017 Dec;155(4):118-130

Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany - Email:

Amyotrophic lateral sclerosis (ALS) is traditionally considered a disease affecting exclusively motor neurons. However, much evidence points towards additional involvement of brain systems other than the motor. As much as half of ALS patients display cognitive-behavioral disturbances. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.12871/00039829201746DOI Listing
December 2017
6 Reads

Target-enriched sequencing of chromosome 17q21.31 in sporadic tauopathies reveals no candidate variants.

Neurobiol Aging 2018 06 11;66:177.e7-177.e10. Epub 2018 Jan 11.

Neurogenetics Laboratory, Division of Neurosciences, Center for Applied Medical Research, University of Navarra (CIMA), Pamplona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas, CIBERNED, Instituto de Salud Carlos III, Madrid, Spain; Movement Disorders Unit, Department of Neurology, Hospital Universitari Mutua de Terrassa, and Fundació per la Recerca Biomèdica i Social Mútua Terrassa, Terrassa, Barcelona, Spain. Electronic address:

The main genetic risk factors for progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are located at chromosome 17q21.31. The identification of risk H1 subhaplotypes suggests that disease-specific variants can be identified by resequencing the 17q21. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neurobiolaging.2017.12.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6769418PMC
June 2018
30 Reads
5.013 Impact Factor

Oscillatory activity in the cortico-basal ganglia-thalamic neural circuits in Parkinson's disease.

Authors:
Arun Singh

Eur J Neurosci 2018 10 8;48(8):2869-2878. Epub 2018 Feb 8.

Department of Neurology, University of Minnesota, Minneapolis, MN, 55455, USA.

Dopamine is an important neurotransmitter that maintains the balance within the basal ganglia between the direct pathway, which promotes movement, and the indirect pathway, which inhibits movement. Degeneration of dopaminergic neurons in the substantia nigra increases the influence of the indirect pathway, resulting in motor dysfunction in Parkinson's disease (PD). The direct and indirect pathways are composed of basal ganglia and thalamic nuclei, which are interconnected via independent parallel loop circuits with cortical areas and often referred to as cortico-basal ganglia-thalamic (CBT) neural circuits. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ejn.13853DOI Listing
October 2018
6 Reads

Apathy in corticobasal degeneration: possible parietal involvement.

Funct Neurol 2017 Oct/Dec;22(4):201-210

Corticobasal degeneration is a rare disorder, which usually consists of a combination of complex movement disorders, apraxia and cortical changes. Its definition is still evolving and in 2013 an international consortium tried to develop new criteria, based on a systematic literature review. Over a long period of time, we carefully selected 23 patients who fulfilled the criteria for a diagnosis of corticobasal degeneration; all had the so-called corticobasal syndrome phenotype, in accordance with Armstrong et al. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.11138/fneur/2017.32.4.201DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5762105PMC
August 2018
9 Reads

Head to head comparison of [F] AV-1451 and [F] THK5351 for tau imaging in Alzheimer's disease and frontotemporal dementia.

Eur J Nucl Med Mol Imaging 2018 03 16;45(3):432-442. Epub 2017 Nov 16.

Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Kangnam-ku, Seoul, 06351, South Korea.

Purpose: Tau accumulation is a core pathologic change in various neurodegenerative diseases including Alzheimer's disease and frontotemporal lobar degeneration-tau. Recently, tau positron emission tomography tracers such as [F] AV-1451 and [F] THK5351 have been developed to detect tau deposition in vivo. In the present study, we performed a head to head comparison of these two tracers in Alzheimer's disease and frontotemporal dementia cases and aimed to investigate which tracers are better suited to image tau in these disorders. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00259-017-3876-0DOI Listing
March 2018
66 Reads