TY - BOOK ID - 8358958 TI - The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy AU - Rüb, Udo. AU - Vonsattel, Jean Paul G. AU - Heinsen, Helmut. AU - Korf, Horst-Werner. PY - 2015 SN - 3319192841 331919285X PB - Cham : Springer International Publishing : Imprint: Springer, DB - UniCat KW - Huntington Disease KW - Dementia KW - Basal Ganglia Diseases KW - Heredodegenerative Disorders, Nervous System KW - Cognition Disorders KW - Chorea KW - Delirium, Dementia, Amnestic, Cognitive Disorders KW - Brain Diseases KW - Dyskinesias KW - Genetic Diseases, Inborn KW - Neurodegenerative Diseases KW - Mental Disorders KW - Nervous System Diseases KW - Movement Disorders KW - Central Nervous System Diseases KW - Congenital, Hereditary, and Neonatal Diseases and Abnormalities KW - Psychiatry and Psychology KW - Diseases KW - Neurology KW - Medicine KW - Health & Biological Sciences KW - Huntington's disease. KW - Huntington's disease KW - Pathogenesis. KW - Chronic progressive chorea KW - Chronic progressive hereditary chorea KW - Degenerative chorea KW - HC (Disease) KW - HD (Disease) KW - Hereditary chorea KW - Huntington chorea KW - Huntington chronic progressive hereditary chorea KW - Huntington disease KW - Huntington's chorea KW - Lund-Huntington chorea KW - Microcellular striatal syndrome KW - Progressive hereditary chorea, Chronic KW - Medicine. KW - Human genetics. KW - Neurosciences. KW - Pathology. KW - Biomedicine. KW - Human Genetics. KW - Disease (Pathology) KW - Medical sciences KW - Medicine, Preventive KW - Neural sciences KW - Neurological sciences KW - Neuroscience KW - Nervous system KW - Genetics KW - Heredity, Human KW - Human biology KW - Physical anthropology KW - Clinical sciences KW - Medical profession KW - Life sciences KW - Pathology KW - Physicians KW - Genetic disorders KW - Degeneration UR - https://www.unicat.be/uniCat?func=search&query=sysid:8358958 AB - 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. ER -