990 results match your criteria Cortical Basal Ganglionic Degeneration


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

Brain 2019 Feb 11. Epub 2019 Feb 11.

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

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http://dx.doi.org/10.1093/brain/awz019DOI Listing
February 2019
6 Reads

Corticobasal Syndrome: Neuroimaging and Neurophysiological Advances.

Eur J Neurol 2019 Feb 5. Epub 2019 Feb 5.

IRCCS Neuromed Institute, Pozzilli, IS.

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 expressing in the 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

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http://dx.doi.org/10.1111/ene.13928DOI Listing
February 2019

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

Neuropathology 2019 Jan 15. 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

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http://dx.doi.org/10.1111/neup.12532DOI Listing
January 2019
2 Reads

Cerebellar degeneration correlates with motor symptoms in Huntington disease.

Ann Neurol 2019 Jan 11. Epub 2019 Jan 11.

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

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http://dx.doi.org/10.1002/ana.25413DOI Listing
January 2019
6 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

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

Regional subcortical shape analysis in premanifest Huntington's disease.

Hum Brain Mapp 2018 Oct 30. 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

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http://dx.doi.org/10.1002/hbm.24456DOI Listing
October 2018
10 Reads

Imaging of Motor Cortex Physiology in Parkinson's Disease.

Mov Disord 2018 Nov 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

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

A Cortical Pathogenic Theory of Parkinson's Disease.

Neuron 2018 Sep;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

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

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

Neurobiol Dis 2018 Nov 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

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http://dx.doi.org/10.1016/j.nbd.2018.07.029DOI Listing
November 2018
4 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

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

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

Neurocase 2018 Jun 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

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

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

Synapse 2018 Dec 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

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http://dx.doi.org/10.1002/syn.22060DOI Listing
December 2018
3 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

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http://dx.doi.org/10.1016/j.nicl.2018.05.031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6008284PMC
January 2019
10 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

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http://dx.doi.org/10.1007/978-1-4939-7825-0_15DOI Listing
January 2018
18 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

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http://dx.doi.org/10.1097/WCO.0000000000000582DOI Listing
August 2018
2 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

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

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

J Neural Transm (Vienna) 2018 Aug 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

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http://link.springer.com/10.1007/s00702-018-1864-6
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http://dx.doi.org/10.1007/s00702-018-1864-6DOI Listing
August 2018
5 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

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http://dx.doi.org/10.1007/s11682-018-9837-9DOI Listing
December 2018
10 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

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http://dx.doi.org/10.12871/00039829201746DOI Listing
December 2017
3 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

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http://dx.doi.org/10.1016/j.neurobiolaging.2017.12.026DOI Listing
June 2018
7 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 Oct 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

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http://dx.doi.org/10.1111/ejn.13853DOI Listing
October 2018
2 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

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August 2018
5 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

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

Grey matter volume loss is associated with specific clinical motor signs in Huntington's disease.

Parkinsonism Relat Disord 2018 Jan 2;46:56-61. Epub 2017 Nov 2.

Department of Neurology, Leiden University Medical Center, PO BOX 9600, 2300 RC Leiden, The Netherlands. Electronic address:

Background: Motor disturbances are clinical hallmarks of Huntington's disease (HD) and involve chorea, dystonia, hypokinesia and visuomotor dysfunction. Investigating the association between specific motor signs and different regional volumes is important to understand the heterogeneity of HD.

Objective: To investigate the motor phenotype of HD and associations with subcortical and cortical grey matter volume loss. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2017.11.001DOI Listing
January 2018
5 Reads

The Effects of Medium Spiny Neuron Morphologcial Changes on Basal Ganglia Network under External Electric Field: A Computational Modeling Study.

Front Comput Neurosci 2017 26;11:91. Epub 2017 Oct 26.

Department of Mathematics and Science, Henan Institute of Science and Technology, Xinxiang, China.

The damage of dopaminergic neurons that innervate the striatum has been considered to be the proximate cause of Parkinson's disease (PD). In the dopamine-denervated state, the loss of dendritic spines and the decrease of dendritic length may prevent medium spiny neuron (MSN) from receiving too much excitatory stimuli from the cortex, thereby reducing the symptom of Parkinson's disease. However, the reduction in dendritic spine density obtained by different experiments is significantly different. Read More

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http://dx.doi.org/10.3389/fncom.2017.00091DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5662631PMC
October 2017
1 Read

Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.

Neuropathology 2018 Jun 7;38(3):281-287. Epub 2017 Nov 7.

Department of Psychiatry, University of Tsukuba, Ibaraki, Japan.

Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Read More

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http://dx.doi.org/10.1111/neup.12442DOI Listing
June 2018
12 Reads

Corticobasal degeneration: key emerging issues.

Authors:
F Ali K A Josephs

J Neurol 2018 Feb 23;265(2):439-445. Epub 2017 Oct 23.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Corticobasal degeneration (CBD) was first described by Rebeiz et al. in 1967, and was called corticodentatonigral degeneration with neuronal achromasia [1]. Since then, our knowledge of the clinical features and underlying tau pathology has grown tremendously. Read More

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http://dx.doi.org/10.1007/s00415-017-8644-3DOI Listing
February 2018

Reduced striato-cortical and inhibitory transcallosal connectivity in the motor circuit of Huntington's disease patients.

Hum Brain Mapp 2018 01 8;39(1):54-71. Epub 2017 Oct 8.

Cognition and Brain Plasticity Unit, IDIBELL (Institut d'Investigació Biomèdica de Bellvitge), L'Hospitalet de Llobregat, Barcelona, Spain.

Huntington's disease (HD) is a neurodegenerative disorder which is primarily associated with striatal degeneration. However, the alterations in connectivity of this structure in HD have been underinvestigated. In this study, we analyzed the functional and structural connectivity of the left putamen, while participants performed a finger-tapping task. Read More

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http://dx.doi.org/10.1002/hbm.23813DOI Listing
January 2018
17 Reads

The Neuropsychology (Broadly Conceived) of Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration.

Arch Clin Neuropsychol 2017 Nov;32(7):861-875

Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA.

Objective: To review the cognitive and behavioral features of the different atypical parkinsonian syndromes in which motor symptoms dominate early clinical symptomology: multiple systems atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The impact of cognitive and behavioral deficits on quality of life, associations between neuropsychological and neuropsychiatric findings and brain imaging, and cognitive and behavioral symptom management are also discussed.

Method: A review of the available MSA, PSP, and CBD literature was conducted, with emphasis given to studies investigating the cognitive and behavioral features of the syndromes. Read More

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http://dx.doi.org/10.1093/arclin/acx093DOI Listing
November 2017
14 Reads

Intrafamilial phenotypic heterogeneity in a Taiwanese family with a MAPT p.R5H mutation: a case report and literature review.

BMC Neurol 2017 Sep 18;17(1):186. Epub 2017 Sep 18.

Department of Neurology, MacKay Memorial Hospital, No. 92, Sec. 2, Zhongshan N. Rd., Zhongshan Dist, Taipei City, 10449, Taiwan.

Background: Frontotemporal degeneration (FTD) is a clinically and genetically heterogeneous neurodegenerative disorder characterized by deficits in executive function that frequently overlaps with parkinsonism and motor neuron disorders. Several genes have been identified to cause autosomal dominant forms of FTD, including the gene coding for the protein associated with microtubule tau (MAPT). While most reported pathogenic mutations in MAPT occur in exons 9-13, few families have been reported with mutations outside of this region. Read More

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http://dx.doi.org/10.1186/s12883-017-0966-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5604294PMC
September 2017
10 Reads

Evidence of semantic processing impairments in behavioural variant frontotemporal dementia and Parkinson's disease.

Curr Opin Neurol 2017 Dec;30(6):617-622

Department of Neurology and Penn Frontotemporal Degeneration Center, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Purpose Of Review: Category-specific impairments caused by brain damage can provide important insights into how semantic concepts are organized in the brain. Recent research has demonstrated that disease to sensory and motor cortices can impair perceptual feature knowledge important to the representation of semantic concepts. This evidence supports the grounded cognition theory of semantics, the view that lexical knowledge is partially grounded in perceptual experience and that sensory and motor regions support semantic representations. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000498DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5909813PMC
December 2017
9 Reads

Loss of Hyperdirect Pathway Cortico-Subthalamic Inputs Following Degeneration of Midbrain Dopamine Neurons.

Neuron 2017 Sep;95(6):1306-1318.e5

Department of Physiology, Feinberg School of Medicine, Northwestern University, 303 E. Chicago Ave, Chicago, IL 60611, USA. Electronic address:

The motor symptoms of Parkinson's disease (PD) are linked to abnormally correlated and coherent activity in the cortex and subthalamic nucleus (STN). However, in parkinsonian mice we found that cortico-STN transmission strength had diminished by 50%-75% through loss of axo-dendritic and axo-spinous synapses, was incapable of long-term potentiation, and less effectively patterned STN activity. Optogenetic, chemogenetic, genetic, and pharmacological interrogation suggested that downregulation of cortico-STN transmission in PD mice was triggered by increased striato-pallidal transmission, leading to disinhibition of the STN and increased activation of STN NMDA receptors. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08966273173078
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http://dx.doi.org/10.1016/j.neuron.2017.08.038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5679443PMC
September 2017
28 Reads

Cognitive dysfunction in corticobasal degeneration.

Arq Neuropsiquiatr 2017 Aug;75(8):570-579

University Health Network, Toronto Western Hospital, Morton and Gloria Shulman Movement Disorders Centre and the Edmond J. Safra Program in Parkinson's Disease, Toronto, ON, M5T 2S8, Canada.

Corticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Read More

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http://dx.doi.org/10.1590/0004-282X20170077DOI Listing
August 2017
4 Reads

Study on Lesion Assessment of Cerebello-Thalamo-Cortical Network in Wilson's Disease with Diffusion Tensor Imaging.

Neural Plast 2017 11;2017:7323121. Epub 2017 Jul 11.

The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei, Anhui 230012, China.

Wilson's disease (WD) is a genetic disorder of copper metabolism with pathological copper accumulation in the brain and any other tissues. This article aimed to assess lesions in cerebello-thalamo-cortical network with an advanced technique of diffusion tensor imaging (DTI) in WD. 35 WD patients and 30 age- and sex-matched healthy volunteers were recruited to accept diffusion-weighted images with 15 gradient vectors and conventional magnetic resonance imaging (MRI). Read More

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http://dx.doi.org/10.1155/2017/7323121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5525080PMC
May 2018
18 Reads

In vivo retention of F-AV-1451 in corticobasal syndrome.

Neurology 2017 Aug 28;89(8):845-853. Epub 2017 Jul 28.

From the Departments of Neurology (R.S., H.W., C.N.), Clinical Neurophysiology (D.H.), Radiation Physics (T.O.), and Clinical Physiology and Nuclear Medicine (J.J.), Skåne University Hospital (D.v.W.), Lund; Clinical Memory Research Unit (R.S., M.S., C.N., O.H.), Department of Clinical Sciences (D.v.W.), and Department of Diagnostic Radiology (D.v.W.), Lund University, Malmö; Wallenberg Centre for Molecular and Translational Medicine and the Department of Psychiatry and Neurochemistry (M.S.), University of Gothenburg; Department of Clinical Neuroscience (P.S.), CMM L8:01, Stockholm; and Memory Clinic (O.H.), Skåne University Hospital, Malmö, Sweden.

Objective: To study the usefulness of F-AV-1451 PET in patients with corticobasal syndrome (CBS).

Methods: We recruited 8 patients with CBS, 17 controls, 31 patients with Alzheimer disease (AD), and 11 patients with progressive supranuclear palsy (PSP) from the Swedish BioFINDER study. All patients underwent clinical assessment, F-AV-1451 PET, MRI, and quantification of β-amyloid pathology. Read More

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http://dx.doi.org/10.1212/WNL.0000000000004264DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5580862PMC
August 2017
40 Reads

Atrophic degeneration of cerebellum impairs both the reactive and the proactive control of movement in the stop signal paradigm.

Exp Brain Res 2017 10 17;235(10):2971-2981. Epub 2017 Jul 17.

Department of Physiology and Pharmacology, Sapienza University of Rome, Piazzale Aldo Moro 5, 00185, Rome, Italy.

The cognitive control of movement suppression, including performance monitoring, is one of the core properties of the executive system. A complex cortical and subcortical network involving cerebral cortex, thalamus, subthalamus, and basal ganglia has been regarded as the neural substrate of inhibition of programmed movements. Using the countermanding task, a suitable tool to explore behavioral components of movement suppression, the contribution of the cerebellum in the proactive control and monitoring of voluntary action has been recently described in patients affected by focal lesions involving in particular the cerebellar dentate nucleus. Read More

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http://dx.doi.org/10.1007/s00221-017-5027-zDOI Listing
October 2017
4 Reads

Voluntary saccade inhibition deficits correlate with extended white-matter cortico-basal atrophy in Huntington's disease.

Neuroimage Clin 2017 9;15:502-512. Epub 2017 Jun 9.

Departamento de Fisiología, Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico. Electronic address:

The ability to inhibit automatic versus voluntary saccade commands in demanding situations can be impaired in neurodegenerative diseases such as Huntington's disease (HD). These deficits could result from disruptions in the interaction between basal ganglia and the saccade control system. To investigate voluntary oculomotor control deficits related to the cortico-basal circuitry, we evaluated early HD patients using an interleaved pro- and anti-saccade task that requires flexible executive control to generate either an automatic response (look at a peripheral visual stimulus) or a voluntary response (look away from the stimulus in the opposite direction). Read More

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http://dx.doi.org/10.1016/j.nicl.2017.06.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5472191PMC
April 2018
10 Reads

Structural MRI correlates of amyotrophic lateral sclerosis progression.

J Neurol Neurosurg Psychiatry 2017 11 13;88(11):901-907. Epub 2017 May 13.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

Purpose: Amyotrophic lateral sclerosis (ALS) presents with varying degrees of brain degeneration that can extend beyond the corticospinal tract (CST). Furthermore, the clinical course and progression of ALS varies widely. Brain degeneration detected using structural MRI could reflect disease progression. Read More

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http://dx.doi.org/10.1136/jnnp-2016-314337DOI Listing
November 2017
14 Reads

Differentiating cognitive impairment due to corticobasal degeneration and Alzheimer disease.

Neurology 2017 Mar 24;88(13):1273-1281. Epub 2017 Feb 24.

From the Charles F. and Joanne Knight Alzheimer Disease Research Center (G.S.D., T.S.L., J.H., A.M.G., E.A.G., C.M.R., N.J.C., J.C.M.), Department of Neurology (G.S.D., T.S.L., J.H., C.M.R., N.J.C., J.C.C.), Division of Biostatistics (E.A.G.), and Department of Pathology and Immunology (N.J.C.), Washington University School of Medicine (G.S.D., T.S.L., J.H., A.M.G., E.A.G., C.M.R., N.J.C., J.C.M.), St. Louis, MO; Ajou University School of Medicine (T.S.L.), Suwon, Republic of Korea; and Icahn School of Medicine at Mount Sinai (A.M.G.), New York, NY.

Objective: To identify clinical features that reliably differentiate individuals with cognitive impairment due to corticobasal degeneration (CBD) and Alzheimer disease (AD).

Methods: Clinical features were compared between individuals with autopsy-proven CBD (n = 17) and AD (n = 16). All individuals presented with prominent cognitive complaints and were evaluated annually with semistructured interviews, detailed neurologic examinations, and neuropsychological testing. Read More

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http://dx.doi.org/10.1212/WNL.0000000000003770DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5373780PMC
March 2017
8 Reads

Compulsive Social Behavior Emerges after Selective Ablation of Striatal Cholinergic Interneurons.

J Neurosci 2017 03 13;37(11):2849-2858. Epub 2017 Feb 13.

Universidad de Buenos Aires. Facultad de Medicina, Departamento de Fisiología, Buenos Aires, Argentina, and CONICET-Universidad de Buenos Aires, Instituto de Fisiología y Biofísica "Bernardo Houssay" (IFIBIO-Houssay), Grupo de Neurociencia de Sistemas, Buenos Aires 1121, Argentina

The mechanisms underlying social dysfunction in neuropsychiatric conditions such as obsessive-compulsive disorder and Tourette syndrome remain uncertain. However, it is known that dysfunctions in basal ganglia, including a reduced number of striatal cholinergic interneurons (SCIN), are involved in their pathophysiology. To explore the role of SCIN in relation to perseverative behaviors, we characterized a new transgenic mouse model in which inducible ablation of SCIN is achieved with high efficiency in a cell-type- and region-specific manner. Read More

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http://dx.doi.org/10.1523/JNEUROSCI.3460-16.2017DOI Listing
March 2017
7 Reads

Longitudinal changes of cortical microstructure in Parkinson's disease assessed with T1 relaxometry.

Neuroimage Clin 2017 21;13:405-414. Epub 2016 Dec 21.

Department of Neurology, Goethe University, Frankfurt/Main, Germany; Brain Imaging Center, Goethe University, Frankfurt/Main, Germany.

Background: Histological evidence suggests that pathology in Parkinson's disease (PD) goes beyond nigrostriatal degeneration and also affects the cerebral cortex. Quantitative MRI (qMRI) techniques allow the assessment of changes in brain tissue composition. However, the development and pattern of disease-related cortical changes have not yet been demonstrated in PD with qMRI methods. Read More

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http://dx.doi.org/10.1016/j.nicl.2016.12.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5226811PMC
November 2017
12 Reads

Retinal thinning is uniquely associated with medial temporal lobe atrophy in neurologically normal older adults.

Neurobiol Aging 2017 03 21;51:141-147. Epub 2016 Dec 21.

Department of Neurology, University of California, San Francisco, CA, USA.

Given the converging pathologic and epidemiologic data indicating a relationship between retinal integrity and neurodegeneration, including Alzheimer's disease (AD), we aimed to determine if retinal structure correlates with medial temporal lobe (MTL) structure and function in neurologically normal older adults. Spectral-domain optical coherence tomography, verbal and visual memory testing, and 3T-magnetic resonance imaging of the brain were performed in 79 neurologically normal adults enrolled in a healthy aging cohort study. Retinal nerve fiber thinning and reduced total macular and macular ganglion cell volumes were each associated with smaller MTL volumes (ps < 0. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2016.12.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5554591PMC
March 2017
47 Reads

Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease.

Elife 2016 12 20;5. Epub 2016 Dec 20.

Department of Physiology, Feinberg School of Medicine, Northwestern University, Chicago, United States.

The subthalamic nucleus (STN) is an element of cortico-basal ganglia-thalamo-cortical circuitry critical for action suppression. In Huntington's disease (HD) action suppression is impaired, resembling the effects of STN lesioning or inactivation. To explore this potential linkage, the STN was studied in BAC transgenic and Q175 knock-in mouse models of HD. Read More

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http://dx.doi.org/10.7554/eLife.21616DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199195PMC
December 2016
1 Read

Presymptomatic anterior frontal involvement in corticobasal degeneration.

Brain 2016 12;139(Pt 12):3059-3062

Division of Neuroscience and Experimental Psychology, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK

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http://dx.doi.org/10.1093/brain/aww267DOI Listing
December 2016
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Multimodal evaluation demonstrates in vivo F-AV-1451 uptake in autopsy-confirmed corticobasal degeneration.

Acta Neuropathol 2016 12 4;132(6):935-937. Epub 2016 Nov 4.

Department of Neurology, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 3 West Gates, Philadelphia, PA, 19104, USA.

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http://dx.doi.org/10.1007/s00401-016-1640-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5154298PMC
December 2016
21 Reads

Astrogliopathy predominates the earliest stage of corticobasal degeneration pathology.

Brain 2016 12 25;139(Pt 12):3237-3252. Epub 2016 Oct 25.

1 Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology, University College London, London, UK

SEE KOBYLECKI AND MANN DOI101093/AWW267 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Animal models have shown that tau seeding and propagation are strain- and neural network-specific. The study of preclinical cases is valuable to gain insights into early pathological features of corticobasal degeneration and its progression. Three preclinical corticobasal degeneration cases and six age-matched end-stage corticobasal degeneration cases were included in this study. Read More

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http://dx.doi.org/10.1093/brain/aww256DOI Listing
December 2016
12 Reads

Zika Virus-Associated Micrencephaly: A Thorough Description of Neuropathologic Findings in the Fetal Central Nervous System.

Arch Pathol Lab Med 2017 Jan 11;141(1):73-81. Epub 2016 Oct 11.

From the Department of Pathology, University Clinical Centre, Maribor, Slovenia (Dr Štrafela); the Institutes of Pathology (Drs Vizjak, Mlakar, Pižem, and Popović and Mrs Mraz) and Microbiology and Immunology (Dr Županc), Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia; and the Department of Perinatology, Division of Gynecology and Obstetrics, University Medical Centre, Ljubljana, Slovenia (Dr Tul).

Context: -The 2015 outbreak of Zika virus in Brazil resulted in a 20-times increased prevalence of congenital microcephaly in stillborns and neonates and was instrumental in raising the suspicion of a causal association between Zika virus and microcephaly.

Objective: -To provide a comprehensive description of the neuropathologic features of congenital Zika virus infection.

Design: -Autopsy evaluation of the brain from a fetus of 32 weeks and 6 days of gestation, with a prenatal diagnosis of microcephaly associated with polymerase chain reaction-confirmed, fetal, Zika virus infection. Read More

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http://dx.doi.org/10.5858/arpa.2016-0341-SADOI Listing
January 2017
24 Reads

In vivo visualization of tau deposits in corticobasal syndrome by 18F-THK5351 PET.

Neurology 2016 Nov 28;87(22):2309-2316. Epub 2016 Oct 28.

From the Departments of Neurology (A.K., T.H., T.B., N.S., R.O., S.Y., J.K., M.E., M.A.), Pharmacology (N.O., R.H., K.Y.), and Diagnostic Radiology (S.M.), Tohoku University Graduate School of Medicine; Divisions of Cyclotron Nuclear Medicine (S.W., K.H., M.T.) and Radiopharmaceutical Chemistry (Y.F., R.I., S.F.), Cyclotron and Radioisotope Center, and Department of Geriatric and Respiratory Medicine (A.I., K.F., H.A.) and Division of Neuroimaging (Y.K.), Institute of Development, Aging and Cancer, Tohoku University; Department of Neurology (M.K.), Tohoku Pharmaceutical University Hospital; Department of Neurology (O.T.), Sendai Medical Center; and Department of Neurology (A.T.), National Hospital Organization, Sendai Nishitaga Hospital, Sendai, Japan.

Objective: To determine whether 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 H-THK5351 in postmortem brain tissues from a patient with corticobasal degeneration (CBD). In clinical PET studies, 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). Read More

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http://dx.doi.org/10.1212/WNL.0000000000003375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5135024PMC
November 2016
76 Reads

Symptom heterogeneity in Huntington's disease correlates with neuronal degeneration in the cerebral cortex.

Neurobiol Dis 2016 Dec 25;96:67-74. Epub 2016 Aug 25.

Centre for Brain Research, University of Auckland, Auckland, New Zealand; Department of Anatomy and Medical Imaging, University of Auckland, Auckland, New Zealand. Electronic address:

Background: Huntington's disease (HD) is characterised by variable symptoms and neuropathology of the basal ganglia and cortex. Previously, we have shown that the pattern of pyramidal cell loss in 8 different cortical regions correlates with the phenotypic variability in HD. In the primary motor and anterior cingulate cortices, the pattern of interneuron degeneration correlates with pyramidal cell death and variable HD symptom profiles. Read More

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http://dx.doi.org/10.1016/j.nbd.2016.08.015DOI Listing
December 2016
5 Reads