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Cortical Basal Ganglionic Degeneration

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Regional brain susceptibility to neurodegeneration: what is the role of glial cells?

Andrea Beatriz Cragnolini, Giorgia Lampitella, Assunta Virtuoso, Immacolata Viscovo, Fivos Panetsos, Michele Papa, Giovanni Cirillo,

The main pathological feature of the neurodegenerative diseases is represented by neuronal death that represents the final step of a cascade of adverse/hostile events. Early in the neurodegenerative process, glial cells (including astrocytes, microglial cells, and oligodendrocytes) activate and trigger an insidious neuroinflammatory reaction, metabolic decay, blood brain barrier dysfunction ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(5):838-842]

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

Effrosyni Koutsouraki, Dimitrios Michmizos, Olga Patsi, John Tzartos, Martha Spilioti, Marianthi Arnaoutoglou, Magda Tsolaki,

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 ... Read more >>

Virol. J. (Virology journal)
[2020, 17(1):35]

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Wallerian degeneration of bilateral cerebral peduncles after acute carbon monoxide poisoning.

Sui-Yi Xu, Chang-Xin Li, Le-Yi Li, Yu Song, Yi Sui,

BACKGROUND:Cases of Wallerian degeneration of bilateral cerebral peduncles after acute carbon monoxide poisoning have not yet been reported. To date, most of the delayed encephalopathy after acute carbon monoxide poisoning (DEACMP) lesions captured in magnetic resonance imaging (MRI) has been located in the subcortical white matter and basal ganglia. Here ... Read more >>

BMC Neurol (BMC neurology)
[2020, 20(1):96]

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Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3.

Po-Shan Wang, Yu-Te Wu, Tzu-Yun Wang, Hsiu-Mei Wu, Bing-Wen Soong, Chi-Wen Jao,

Background: 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. Method: We used ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:124]

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Fabry Disease With Concomitant Lewy Body Disease.

Kelly Del Tredici, Albert C Ludolph, Simone Feldengut, Christian Jacob, Heinz Reichmann, Jürgen R Bohl, Heiko Braak,

Although Gaucher disease can be accompanied by Lewy pathology (LP) and extrapyramidal symptoms, it is unknown if LP exists in Fabry disease (FD), another progressive multisystem lysosomal storage disorder. We aimed to elucidate the distribution patterns of FD-related inclusions and LP in the brain of a 58-year-old cognitively unimpaired male ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):378-392]

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Neuroimaging Biomarkers in SCA2 Gene Carriers.

Mario Mascalchi, Alessandra Vella,

A variety of Magnetic Resonance (MR) and nuclear medicine (NM) techniques have been used in symptomatic and presymptomatic SCA2 gene carriers to explore,in vivo, the physiopathological biomarkers of the neurological dysfunctions characterizing the associated progressive disease that presents with a cerebellar syndrome, or less frequently, with a levodopa-responsive parkinsonian syndrome. ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(3):]

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Pseudotumour Cerebri Syndrome in China: A Cohort Study.

Qian Chen, Chaoyi Feng, Guixian Zhao, Weimin Chen, Min Wang, Xinghuai Sun, Yan Sha, Zhenxin Li, Guohong Tian,

Pseudotumour cerebri syndrome (PTCS) remains to be fully investigated in Chinese patients and our study reported PTCS-related clinical differences between Chinese patients and Western patients. This study enrolled 55 consecutive patients (females: 44, median age: 37 y, age range: 14-62 y) with PTCS diagnosed from October 2015 to December 2017. Nine (16.4%, ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):1222]

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A Clinicopathological Report of a 93-Year-Old Former Street Boxer With Coexistence of Chronic Traumatic Encephalopathy, Alzheimer's Disease, Dementia With Lewy Bodies, and Hippocampal Sclerosis With TDP-43 Pathology.

Chunhui Yang, Sukriti Nag, Guoqiang Xing, Neelum T Aggarwal, Julie A Schneider,

Chronic traumatic encephalopathy (CTE) was recently recognized as a new tauopathy in which multifocal perivascular phosphorylated tau aggregates accumulate in neurons, astrocytes, and neurites at the depths of the cortical sulci. Traumatic brain injury (TBI) in early or mid-life is known to be associated with an increased risk of dementia ... Read more >>

Front Neurol (Frontiers in neurology)
[2020, 11:42]

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MS optic neuritis-induced long-term structural changes within the visual pathway.

Marc Pawlitzki, Marc Horbrügger, Kristian Loewe, Jörn Kaufmann, Roland Opfer, Markus Wagner, Khaldoon O. Al-Nosairy, Sven G. Meuth, Michael B. Hoffmann, Sven Schippling,

BackgroundThe visual pathway is commonly involved in multiple sclerosis (MS), even in its early stages, including clinical episodes of optic neuritis (ON). The long-term structural damage within the visual compartment in patients with ON, however, is yet to be elucidated.ObjectiveOur aim was to characterize visual system structure abnormalities using MRI ... Read more >>

(Neurology® Neuroimmunology & Neuroinflammation)
[2020, 7(2):]

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Reflections on the Utility of the Retina as a Biomarker for Alzheimer's Disease: A Literature Review.

Jennifer Ngolab, Patrick Honma, Robert A Rissman,

As a part of the central nervous system, the retina may reflect both physiologic processes and abnormalities related to diseases of the brain. Indeed, a concerted effort has been put forth to understand how Alzheimer's disease (AD) pathology may manifest in the retina as a means to assess the state ... Read more >>

Neurol Ther (Neurology and therapy)
[2019, 8(Suppl 2):57-72]

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Expression of deubiquitinating enzyme genes in the developing mammal retina.

Mariona Esquerdo-Barragán, Matthew J Brooks, Vasileios Toulis, Anand Swaroop, Gemma Marfany,

Purpose:Genes involved in the development and differentiation of the mammalian retina are also associated with inherited retinal dystrophies (IRDs) and age-related macular degeneration. Transcriptional regulation of retinal cell differentiation has been addressed by genetic and transcriptomic studies. Much less is known about the posttranslational regulation of key regulatory proteins, although ... Read more >>

Mol. Vis. (Molecular vision)
[2019, 25:800-813]

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Nafamostat and sepimostat identified as novel neuroprotective agents via NR2B N-methyl-D-aspartate receptor antagonism using a rat retinal excitotoxicity model.

Masahiro Fuwa, Masaaki Kageyama, Koji Ohashi, Masaaki Sasaoka, Ryuichi Sato, Masami Tanaka, Kei Tashiro,

In addition to its role in the treatment of pancreatitis, the serine protease inhibitor nafamostat exhibits a retinal protective effect. However, the exact mechanisms underlying this effect are unknown. In this study, the neuroprotective effects of nafamostat and its orally active derivative sepimostat against excitotoxicity were further characterised in vitro ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):20409]

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Residual Visual Responses in Patients With Retinitis Pigmentosa Revealed by Functional Magnetic Resonance Imaging.

Elisa Castaldi, Guido Marco Cicchini, Benedetto Falsini, Paola Binda, Maria Concetta Morrone,

Purpose:We evaluated the potential of magnetic resonance imaging in identifying signs of cortical visual processing with greater sensitivity than standard ophthalmological measures in patients with retinitis pigmentosa (RP) at advanced stages. Methods:Eight patients affected with RP with only bare light perception and weak or absent visual evoked potential (VEP) or ... Read more >>

Transl Vis Sci Technol (Translational vision science & technology)
[2019, 8(6):44]

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Research progress on the role of type I vesicular glutamate transporter (VGLUT1) in nervous system diseases.

Xianchao Du, Jiashuo Li, Minghui Li, Xinxin Yang, Zhipeng Qi, Bin Xu, Wei Liu, Zhaofa Xu, Yu Deng,

Glutamate (Glu) is the predominant excitatory neurotransmitter in the central nervous system (CNS). Glutamatergic transmission is critical for controlling neuronal activity. In presynaptic neurons, Glu is stored in synaptic vesicles and released by stimulation. The homeostasis of glutamatergic system is maintained by a set of transporters in the membrane of ... Read more >>

Cell Biosci (Cell & bioscience)
[2020, 10:26]

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Manganese-Enhanced Magnetic Resonance Imaging: Application in Central Nervous System Diseases.

Jun Yang, Qinqing Li,

Manganese-enhanced magnetic resonance imaging (MEMRI) relies on the strong paramagnetism of Mn2+. Mn2+ is a calcium ion analog and can enter excitable cells through voltage-gated calcium channels. Mn2+ can be transported along the axons of neurons via microtubule-based fast axonal transport. Based on these properties, MEMRI is used to describe ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:143]

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A Rare Mutation in The APOB Gene Associated with Neurological Manifestations in Familial Hypobetalipoproteinemia.

Joanna Musialik, Anna Boguszewska-Chachulska, Dorota Pojda-Wilczek, Agnieszka Gorzkowska, Robert Szymańczak, Magdalena Kania, Agata Kujawa-Szewieczek, Małgorzata Wojcieszyn, Marek Hartleb, Andrzej Więcek,

Clinical phenotypes of familial hypobetalipoproteinemia (FHBL) are related to a number of defective apolipoprotein B (APOB) alleles. Fatty liver disease is a typical manifestation, but serious neurological symptoms can appear. In this study, genetic analysis of the APOB gene and ophthalmological diagnostics were performed for family members with FHBL. Five ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(4):]

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Cortical and Striatal Circuits in Huntington's Disease.

Sonja Blumenstock, Irina Dudanova,

Huntington's disease (HD) is a hereditary neurodegenerative disorder that typically manifests in midlife with motor, cognitive, and/or psychiatric symptoms. The disease is caused by a CAG triplet expansion in exon 1 of the huntingtin gene and leads to a severe neurodegeneration in the striatum and cortex. Classical electrophysiological studies in ... Read more >>

Front Neurosci (Frontiers in neuroscience)
[2020, 14:82]

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The Cortico-Basal Ganglia-Cerebellar Network: Past, Present and Future Perspectives.

Demetrio Milardi, Angelo Quartarone, Alessia Bramanti, Giuseppe Anastasi, Salvatore Bertino, Gianpaolo Antonio Basile, Piero Buonasera, Giorgia Pilone, Giuseppe Celeste, Giuseppina Rizzo, Daniele Bruschetta, Alberto Cacciola,

Much of our present understanding of the function and operation of the basal ganglia rests on models of anatomical connectivity derived from tract-tracing approaches in rodents and primates. However, the last years have been characterized by promising step forwards in the in vivo investigation and comprehension of brain connectivity in ... Read more >>

Front Syst Neurosci (Frontiers in systems neuroscience)
[2019, 13:61]

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Neuroimaging in Vascular Parkinsonism.

Karen K Y Ma, Shi Lin, Vincent C T Mok,

PURPOSE OF REVIEW:Being a disease with heterogeneous presentations and unclear consensus on its diagnostic criteria, it is difficult to differentiate vascular parkinsonism (VaP) from other neurodegenerative parkinsonism variants. Ongoing research on structural and functional neuroimaging targeting dopaminergic pathway provides us more insight into the pathophysiology of VaP to improve diagnostic ... Read more >>

Curr Neurol Neurosci Rep (Current neurology and neuroscience reports)
[2019, 19(12):102]

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Targeting Neurovascular Interaction in Retinal Disorders.

Zhongjie Fu, Ye Sun, Bertan Cakir, Yohei Tomita, Shuo Huang, Zhongxiao Wang, Chi-Hsiu Liu, Steve S Cho, William Britton, Timothy S Kern, David A Antonetti, Ann Hellström, Lois E H Smith,

The tightly structured neural retina has a unique vascular network comprised of three interconnected plexuses in the inner retina (and choroid for outer retina), which provide oxygen and nutrients to neurons to maintain normal function. Clinical and experimental evidence suggests that neuronal metabolic needs control both normal retinal vascular development ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(4):]

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The primate model for understanding and restoring vision.

Serge Picaud, Deniz Dalkara, Katia Marazova, Olivier Goureau, Botond Roska, José-Alain Sahel,

Retinal degenerative diseases caused by photoreceptor cell death are major causes of irreversible vision loss. As only primates have a macula, the nonhuman primate (NHP) models have a crucial role not only in revealing biological mechanisms underlying high-acuity vision but also in the development of therapies. Successful translation of basic ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2019, :]

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Gyriform restricted diffusion in adults: looking beyond thrombo-occlusions.

Vivek Pai, Yih Yian Sitoh, Bela Purohit,

Gyriform restricted diffusion (GRD) refers to hyperintense signal involving the cerebral cortex on diffusion-weighted images (DWI) with corresponding hypointensity on apparent diffusion coefficient (ADC) images. These changes are commonly seen following a vascular occlusion, reflecting the limitation of water molecule movement across cell membranes (restricted diffusion) due to the failure ... Read more >>

Insights Imaging (Insights into imaging)
[2020, 11(1):20]

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

Helen Ling, Ellen Gelpi, Karen Davey, Zane Jaunmuktane, Kin Y Mok, Edwin Jabbari, Roberto Simone, Lea R'Bibo, Sebastian Brandner, Matthew J Ellis, Johannes Attems, David Mann, Glenda M Halliday, S Al-Sarraj, J Hedreen, James W Ironside, Gabor G Kovacs, E Kovari, S Love, Jean Paul G Vonsattel, Kieren S J Allinson, Daniela Hansen, Teisha Bradshaw, Núria Setó-Salvia, Selina Wray, Rohan de Silva, Huw R Morris, Thomas T Warner, John Hardy, Janice L Holton, Tamas Revesz,

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. Of the 124 autopsy-confirmed corticobasal degeneration cases collected from 14 centres, we identified 6 RP-CBD cases (4.8%) who ... Read more >>

Acta Neuropathol. (Acta neuropathologica)
[2020, 139(4):717-734]

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The neurology clinic needs monkey research.

Michael E Goldberg,

This report discusses how a number of currently incurable diseases might be treated by advances developed as the result of current ongoing research on monkeys. The diseases discussed include Parkinson's disease, amyotrophic lateral sclerosis, spinal cord injury, peripheral neuropathy, and stroke. Finally, the report discusses the devastating effect the animal ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2019, :]

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Subcellular Abnormalities of Vestibular Nerve Morphology in Patients With Intractable Meniere's Disease.

Pengjun Wang, Huaming Zhu, Wen Lu, Qiang Song, Zhengnong Chen, Yaqin Wu, Hui Wang, Dongzhen Yu, Haibo Ye, Haibo Shi, Haibo Shi, Shankai Yin,

Objective: Few studies so far have focused on the retrocochlear lesions in Meniere's disease (MD). This study aims to investigate pathological alterations in the central portion of the vestibular nerve (VN) in patients with intractable Meniere's disease (MD) and to explore retrocochlear lesions and their relationship with disease severity. Methods: ... Read more >>

Front Neurol (Frontiers in Neurology)
[2019, 10:948]

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