The Experts below are selected from a list of 143253 Experts worldwide ranked by ideXlab platform
Kurt A. Jellinger - One of the best experts on this subject based on the ideXlab platform.
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Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update. II. Hyperkinetic disorders
Journal of Neural Transmission, 2019Co-Authors: Kurt A. JellingerAbstract:Extrapyramidal movement disorders comprise hypokinetic-rigid and hyperkinetic or mixed forms, most of them originating from dysfunction of the basal ganglia (BG) and their information circuits that have been briefly reviewed in part 1 of the papers on neuropathology and pathogenesis of extrapyramidal movement disorders. The classification of hyperkinetic forms distinguishes the following: (1) chorea and related syndromes; (2) dystonias (dyskinesias); (3) tics and tourette disorders; (4) ballism; (5) myoclonic and startle disorders; and (6) tremor syndromes. Recent genetic and molecular classification distinguishes the following: (1) polyglutamine disorders (Huntington’s disease and related disorders); (2) pantothenate kinase associated Neurodegeneration; (3) Wilson’s disease and related disorders; and (4) other hereditary Neurodegenerations without hitherto detected genetic or specific markers. The diversity of phenotypes is related to the deposition of pathologic proteins in distinct cell populations, causing Neurodegeneration due to genetic and environmental factors, but there is frequent overlap between various disorders. Their etiopathogenesis is still poorly understood but is suggested to result from an interaction between genetic and environmental factors, multiple etiologies, and noxious factors (protein mishandling, mitochondrial dysfunction, oxidative stress, excitotoxicity, energy failure, chronic neuroinflammation), being more likely than one single factor. Current clinical consensus criteria have increased the diagnostic accuracy of most neurodegenerative movement disorders, but for their definite diagnosis, histopathological confirmation is required. A timely overview of the neuropathology and pathogenesis of the major hyperkinetic movement disorders is presented.
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Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update—I. Hypokinetic-rigid movement disorders
Journal of Neural Transmission, 2019Co-Authors: Kurt A. JellingerAbstract:Extrapyramidal movement disorders include hypokinetic rigid and hyperkinetic or mixed forms, most of them originating from dysfunction of the basal ganglia (BG) and their information circuits. The functional anatomy of the BG, the cortico-BG–thalamocortical, and BG–cerebellar circuit connections are briefly reviewed. Pathophysiologic classification of extrapyramidal movement disorder mechanisms distinguish (1) parkinsonian syndromes, (2) chorea and related syndromes, (3) dystonias, (4) myoclonic syndromes, (5) ballism, (6) tics, and (7) tremor syndromes. Recent genetic and molecular–biologic classifications distinguish (1) synucleinopathies (Parkinson’s disease, dementia with Lewy bodies, Parkinson’s disease–dementia, and multiple system atrophy); (2) tauopathies (progressive supranuclear palsy, corticobasal degeneration, FTLD-17; Guamian Parkinson–dementia; Pick’s disease, and others); (3) polyglutamine disorders (Huntington’s disease and related disorders); (4) pantothenate kinase-associated Neurodegeneration; (5) Wilson’s disease; and (6) other hereditary Neurodegenerations without hitherto detected genetic or specific markers. The diversity of phenotypes is related to the deposition of pathologic proteins in distinct cell populations, causing Neurodegeneration due to genetic and environmental factors, but there is frequent overlap between various disorders. Their etiopathogenesis is still poorly understood, but is suggested to result from an interaction between genetic and environmental factors. Multiple etiologies and noxious factors (protein mishandling, mitochondrial dysfunction, oxidative stress, excitotoxicity, energy failure, and chronic neuroinflammation) are more likely than a single factor. Current clinical consensus criteria have increased the diagnostic accuracy of most neurodegenerative movement disorders, but for their definite diagnosis, histopathological confirmation is required. We present a timely overview of the neuropathology and pathogenesis of the major extrapyramidal movement disorders in two parts, the first one dedicated to hypokinetic-rigid forms and the second to hyperkinetic disorders.
Brian E. Leonard - One of the best experts on this subject based on the ideXlab platform.
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Neurodegeneration, Neuroregeneration, and Neuroprotection in Psychiatric Disorders.
Modern trends in pharmacopsychiatry, 2017Co-Authors: Siu W. Tang, Daiga M. Helmeste, Brian E. LeonardAbstract:Prevention of deterioration of brain function over time is important in the long-term management of chronic brain disorders such as dementia, schizophrenia, and mood disorders. Although the possibility of neurogenesis in the adult human brain is attractive, and there are psychiatric drugs proven to be effective inducers of neurogenesis in animals, we have yet to see their utility in clinical practice. The terms Neurodegeneration and neuroregeneration are often used in a nonspecific manner. Neuroregeneration may mean neurogenesis, dendritogenesis, spinogenesis, or axonogenesis. The term "neuroprotection" is attractive clinically and may involve different mechanisms. Many causative and protective factors of Neurodegeneration and neuroregeneration have been proposed. However, the specificity of these factors and agents and differential neuronal vulnerability factors have generally been ignored in past studies. It is also hard to separate disease-modifying from "neuroprotective" effects of a drug. The application of stringent long-term neuroanatomical, neurochemical, neurophysiological, and therapeutic efficacy criteria should improve future research in this important area.
Todd E. Golde - One of the best experts on this subject based on the ideXlab platform.
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Zoom in on Neurodegeneration
Molecular Neurodegeneration, 2006Co-Authors: Todd E. GoldeAbstract:Despite the existence of several scientific journals that publish research papers and reviews related to neurodegenerative diseases, a journal specifically devoted to the molecular and cellular aspects of disease mechanisms is lacking. Molecular Neurodegeneration is an open-access, peer-reviewed, online journal created to publish original research articles that address i) the mechanisms of Neurodegeneration at the cellular, subcellular and molecular levels and ii) potential therapeutic interventions for neurodegenerative diseases. Through publication of reviews, editorial commentaries, and meeting reports, Molecular Neurodegeneration will also provide a forum to enhance the exchange of ideas and promote debate that is essential for scientific progress. Molecular Neurodegeneration will enable scientists to rapidly communicate their important research discoveries to their colleagues around the world.
Mark P Mattson - One of the best experts on this subject based on the ideXlab platform.
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calcium and Neurodegeneration
Aging Cell, 2007Co-Authors: Mark P MattsonAbstract:When properly controlled, Ca2+ fluxes across the plasma membrane and between intracellular compartments play critical roles in fundamental functions of neurons, including the regulation of neurite outgrowth and synaptogenesis, synaptic transmission and plasticity, and cell survival. During aging, and particularly in neurodegenerative disorders, cellular Ca2+-regulating systems are compromised resulting in synaptic dysfunction, impaired plasticity and neuronal degeneration. Oxidative stress, perturbed energy metabolism and aggregation of disease-related proteins (amyloid beta-peptide, alpha-synuclein, huntingtin, etc.) adversely affect Ca2+ homeostasis by mechanisms that have been elucidated recently. Alterations of Ca2+-regulating proteins in the plasma membrane (ligand- and voltage-gated Ca2+ channels, ion-motive ATPases, and glucose and glutamate transporters), endoplasmic reticulum (presenilin-1, Herp, and ryanodine and inositol triphosphate receptors), and mitochondria (electron transport chain proteins, Bcl-2 family members, and uncoupling proteins) are implicated in age-related neuronal dysfunction and disease. The adverse effects of aging on neuronal Ca2+ regulation are subject to modification by genetic (mutations in presenilins, alpha-synuclein, huntingtin, or Cu/Zn-superoxide dismutase; apolipoprotein E isotype, etc.) and environmental (dietary energy intake, exercise, exposure to toxins, etc.) factors that may cause or affect the risk of neurodegenerative disease. A better understanding of the cellular and molecular mechanisms that promote or prevent disturbances in cellular Ca2+ homeostasis during aging may lead to novel approaches for therapeutic intervention in neurological disorders such as Alzheimer's and Parkinson's diseases and stroke.
Rafael Simó - One of the best experts on this subject based on the ideXlab platform.
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Neurodegeneration in diabetic retinopathy: does it really matter?
Diabetologia, 2018Co-Authors: Rafael Simó, Alan W. Stitt, Thomas W. GardnerAbstract:The concept of diabetic retinopathy as a microvascular disease has evolved, in that it is now considered a more complex diabetic complication in which Neurodegeneration plays a significant role. In this article we provide a critical overview of the role of microvascular abnormalities and Neurodegeneration in the pathogenesis of diabetic retinopathy. A special emphasis is placed on the pathophysiology of the neurovascular unit (NVU), including the contributions of microvascular and neural elements. The potential mechanisms linking retinal Neurodegeneration and early microvascular impairment, and the effects of neuroprotective drugs are summarised. Additionally, we discuss how the assessment of retinal Neurodegeneration could be an important index of cognitive status, thus helping to identify individuals at risk of dementia, which will impact on current procedures for diabetes management. We conclude that glial, neural and microvascular dysfunction are interdependent and essential for the development of diabetic retinopathy. Despite this intricate relationship, retinal Neurodegeneration is a critical endpoint and neuroprotection, itself, can be considered a therapeutic target, independently of its potential impact on microvascular disease. In addition, interventional studies targeting pathogenic pathways that impact the NVU are needed. Findings from these studies will be crucial, not only for increasing our understanding of diabetic retinopathy, but also to help to implement a timely and efficient personalised medicine approach for treating this diabetic complication.
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Neurodegeneration in diabetic retinopathy: Current concepts and therapeutic implications
Avances en Diabetología, 2014Co-Authors: Cristina Hernández, Rafael SimóAbstract:Abstract Diabetic retinopathy (DR), the most common complication of diabetes and one of the leading causes of preventable blindness, has been considered to be a microcirculatory disease of the retina. However, there is emerging evidence to suggest that retinal Neurodegeneration is an early event in the pathogenesis of DR, which participates in the development of microvascular abnormalities. Therefore, the study of the underlying mechanisms leading to Neurodegeneration and the identification of the mediators linking Neurodegeneration and microangiopathy will be essential for the development of new therapeutic strategies in the early stages of DR. In this review the mechanisms involved in Neurodegeneration, as well as the link between Neurodegeneration and microangiopathy have been updated. Finally, the therapeutic implications and new perspectives based on identifying those patients with retinal Neurodegeneration are presented.
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Neurodegeneration in the diabetic eye: new insights and therapeutic perspectives
Trends in endocrinology and metabolism: TEM, 2013Co-Authors: Rafael Simó, Cristina HernándezAbstract:Diabetic retinopathy (DR), one of the leading causes of preventable blindness, has been considered a microcirculatory disease of the retina. However, there is emerging evidence to suggest that retinal Neurodegeneration is an early event in the pathogenesis of DR, which participates in the development of microvascular abnormalities. Therefore, the study of the underlying mechanisms leading to Neurodegeneration and the identification of the mediators in the crosstalk between Neurodegeneration and microangiopathy will be essential for the development of new therapeutic strategies. In this review, an updated discussion of the mechanisms involved in Neurodegeneration, as well as the link between Neurodegeneration and microangiopathy, is presented. Finally, the therapeutic implications and new perspectives based on identifying those patients with retinal Neurodegeneration are given.
