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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.
Huntington Disease --- Dementia --- Basal Ganglia Diseases --- Heredodegenerative Disorders, Nervous System --- Cognition Disorders --- Chorea --- Delirium, Dementia, Amnestic, Cognitive Disorders --- Brain Diseases --- Dyskinesias --- Genetic Diseases, Inborn --- Neurodegenerative Diseases --- Mental Disorders --- Nervous System Diseases --- Movement Disorders --- Central Nervous System Diseases --- Congenital, Hereditary, and Neonatal Diseases and Abnormalities --- Psychiatry and Psychology --- Diseases --- Neurology --- Medicine --- Health & Biological Sciences --- Huntington's disease. --- Huntington's disease --- Pathogenesis. --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Medicine. --- Human genetics. --- Neurosciences. --- Pathology. --- Biomedicine. --- Human Genetics. --- Disease (Pathology) --- Medical sciences --- Medicine, Preventive --- Neural sciences --- Neurological sciences --- Neuroscience --- Nervous system --- Genetics --- Heredity, Human --- Human biology --- Physical anthropology --- Clinical sciences --- Medical profession --- Life sciences --- Pathology --- Physicians --- Genetic disorders --- Degeneration
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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.
Human genetics --- Neuropathology --- Pathology --- neurologie --- medische genetica --- pathologie --- genetica
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