The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy PDF Download
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Author: Udo Rüb
Publisher: Springer
ISBN: 331919285X
Category : Medical
Languages : en
Pages : 154
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Book Description
This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.
Author: Udo Rüb
Publisher: Springer
ISBN: 331919285X
Category : Medical
Languages : en
Pages : 154
Get Book Here
Book Description
This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.
Author: Hoa Huu Phuc Nguyen
Publisher: Springer
ISBN: 366246344X
Category : Medical
Languages : en
Pages : 402
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Book Description
Motor dysfunction and cognitive impairment are major symptoms in both Huntington’s Disease (HD) and Parkinson’s Disease (PD). A breakthrough in HD research was the identification of the gene that causes this devastating monogenetic illness. Similarly, several genes were found to cause familial forms of PD. With their identification, a plethora of genetic animal models has been generated and has revolutionized the understanding of the pathobiology and pathophysiology of these disorders. The models allow us to study the earliest manifestations of the diseases behaviorally and neuropathologically and help us understand how they progress over time. Additionally, neurotoxic animal models are still of high interest to the PD field, as they are being used to study e.g. mitochondrial dysfunction in PD. This book focuses on animal models of both diseases and how they have helped and will continue to help understand the behavioral neurobiology in these disorders.
Author: Oliver Quarrell
Publisher:
ISBN: 9780192629302
Category : Family & Relationships
Languages : en
Pages : 164
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Book Description
Huntington's Disease is a genetically inherited condition, the result of severe nerve-cell damage in the brain. Due to the recent identification of the gene involved, and the debilitating nature of the disease, a great many more people are now affected either directly or indirectly (familiesand carers) by this condition. The majority of people develop the disease between the ages of 35 and 55, so for those that are aware of a genetic inheritance, there are enormous problems to confront - should you carry on life as normal? Should you start a family? In this, the first book onHuntington's disease written for sufferers and their families, advice is given on living with this disabling illness. Written as much for carers as for the patients themselves, the book aims to answer some of the questions that both sufferer and carer might have. With the identification of theresponsible gene, genetic counselling is now available for those at risk of developing the disease. Though some may wish not to use these services, the book clearly explains the role of the counsellor, and what help is additionally available from the various patient organisations worldwide.
Author: Michael J. Zigmond
Publisher: Elsevier
ISBN: 0123982804
Category : Science
Languages : en
Pages : 823
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Book Description
Neurobiology of Brain Disorders is the first book directed primarily at basic scientists to offer a comprehensive overview of neurological and neuropsychiatric disease. This book links basic, translational, and clinical research, covering the genetic, developmental, molecular, and cellular mechanisms underlying all major categories of brain disorders. It offers students, postdoctoral fellows, and researchers in the diverse fields of neuroscience, neurobiology, neurology, and psychiatry the tools they need to obtain a basic background in the major neurological and psychiatric diseases, and to discern connections between basic research and these relevant clinical conditions. This book addresses developmental, autoimmune, central, and peripheral neurodegeneration; infectious diseases; and diseases of higher function. The final chapters deal with broader issues, including some of the ethical concerns raised by neuroscience and a discussion of health disparities. Included in each chapter is coverage of the clinical condition, diagnosis, treatment, underlying mechanisms, relevant basic and translational research, and key unanswered questions. Written and edited by a diverse team of international experts, Neurobiology of Brain Disorders is essential reading for anyone wishing to explore the basic science underlying neurological and neuropsychiatric diseases. - Links basic, translational, and clinical research on disorders of the nervous system, creating a format for study that will accelerate disease prevention and treatment - Covers a vast array of neurological disorders, including ADHD, Down syndrome, autism, muscular dystrophy, diabetes, TBI, Parkinson, Huntington, Alzheimer, OCD, PTSD, schizophrenia, depression, and pain - Illustrated in full color - Each chapter provides in-text summary points, special feature boxes, and research questions - Provides an up-to-date synthesis of primary source material
Author: Peggy C. Nopoulos
Publisher: MDPI
ISBN: 3039438115
Category : Science
Languages : en
Pages : 102
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Book Description
The Special Issue “Juvenile Onset Huntington’s Disease” highlights the growing interest in understanding the unique aspects of this ultra-rare disorder. After decades of research, gene therapy trials are underway for Adult Onset Huntington’s Disease (AOHD). However, patients with Juvenile Onset Huntington’s Disease (JOHD) are often excluded from these efforts, leaving many questions regarding its phenomenology. The current issue includes seven articles spanning work on the difficult emotional experiences of parents of children with JOHD; a review of the clinical manifestations of JOHD; behavioral issues in JOHD; CAG repeat and age of motor onset; autonomic nervous system dysfunction; and abnormality in MRI metabolic markers. Finally, a review of the therapeutic advances is included, highlighting future possibilities of clinical trials in JOHD subjects. The HD community—patients, family members at-risk for HD, caregivers, health-care professionals and scientists—is keen on expanding our understanding of JOHD. In the flurry of research on AOHD, those with JOHD were seemingly ‘left behind.’ The study of patients who are afflicted early in life with HD has become imperative, with this Special Issue representing just the beginning of the required effort to address this urgent need.
Author: Krista Lanctot
Publisher: Oxford University Press
ISBN: 0198841809
Category : Medical
Languages : en
Pages : 301
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Book Description
Apathy is characterized by loss of motivation, decreased initiative, and emotional blunting. It is highly prevalent in neurological, and psychiatric disorders like Alzheimer's disease, traumatic brain injury, schizophrenia, Parkinson's disease, Huntington's disease, cerebrovascular disorders, and mild behavioural impairment. It has negative outcomes including impairments in activities of daily living, caregiver burden, and higher rates of institutionalization and mortality. The definition of apathy has changed over the years alongside the development of diagnostic criteria and apathy scales and measurements. Apathy is emerging as a treatment target with interest in pharmacological, non-pharmacological, and neuromodulatory treatments for apathy. There is also an increased understanding of the neurobiology of apathy with functional and structural neuroimaging research studies. This book is a comprehensive, in-depth review from experts in neurology and psychiatry. It reviews the current state of apathy in these various disorders while also summarizing apathy diagnostic criteria, scales and measurements, neuropathology, and treatments.
Author: Oliver Quarrell
Publisher: Oxford University Press, USA
ISBN: 0199236127
Category : Medical
Languages : en
Pages : 222
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Book Description
Huntington's disease (HD) is an inherited progressive neurodegenerative disorder. Although onset of HD usually occurs in adulthood, a small percentage of cases develop symptoms before 20 years of age (juvenile-onset Huntington's Disease or JHD). This book summarises, for the first time, the clinical and scientific knowledge available on JHD.
Author: Clévio Nóbrega
Publisher: Springer
ISBN: 3319717790
Category : Medical
Languages : en
Pages : 467
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Book Description
This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies’ development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington’s disease (HD); spinal–bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17). The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.
Author: Institute of Medicine
Publisher: National Academies Press
ISBN: 0309292492
Category : Medical
Languages : en
Pages : 107
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Book Description
Improving and Accelerating Therapeutic Development for Nervous System Disorders is the summary of a workshop convened by the IOM Forum on Neuroscience and Nervous System Disorders to examine opportunities to accelerate early phases of drug development for nervous system drug discovery. Workshop participants discussed challenges in neuroscience research for enabling faster entry of potential treatments into first-in-human trials, explored how new and emerging tools and technologies may improve the efficiency of research, and considered mechanisms to facilitate a more effective and efficient development pipeline. There are several challenges to the current drug development pipeline for nervous system disorders. The fundamental etiology and pathophysiology of many nervous system disorders are unknown and the brain is inaccessible to study, making it difficult to develop accurate models. Patient heterogeneity is high, disease pathology can occur years to decades before becoming clinically apparent, and diagnostic and treatment biomarkers are lacking. In addition, the lack of validated targets, limitations related to the predictive validity of animal models - the extent to which the model predicts clinical efficacy - and regulatory barriers can also impede translation and drug development for nervous system disorders. Improving and Accelerating Therapeutic Development for Nervous System Disorders identifies avenues for moving directly from cellular models to human trials, minimizing the need for animal models to test efficacy, and discusses the potential benefits and risks of such an approach. This report is a timely discussion of opportunities to improve early drug development with a focus toward preclinical trials.
Author: Margaret M. Esiri
Publisher: Cambridge University Press
ISBN: 9780521819152
Category : Medical
Languages : en
Pages : 600
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Book Description
Completely rewritten and updated, this new edition is almost twice the size of its predecessor. Illustrated in colour throughout, and with contributions from the world's leading authorities, it is the definitive reference on the neuropathology of dementia. It gives practical guidance to pathologists, describes the contribution of neuroimaging to diagnosis, and surveys the clinical features of dementia. New material includes: Three entirely new chapters on neuroimaging, molecular diagnostics, and transgenic models. Two chapters on tauopathies under new authorship. A chapter under new authorship on synucleinopathies, which includes multiple system atrophy.
