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008 170212s2015 gw | s |||| 0|eng d
020 _a9783319192857
_9978-3-319-19285-7
024 7 _a10.1007/978-3-319-19285-7
_2doi
035 _ato000560051
040 _aSpringer
_cSpringer
_dRU-ToGU
050 4 _aRC321-580
072 7 _aPSAN
_2bicssc
072 7 _aMED057000
_2bisacsh
082 0 4 _a612.8
_223
100 1 _aRüb, Udo.
_eauthor.
_9466733
245 1 4 _aThe Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy
_helectronic resource
_cby Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf.
250 _a1st ed. 2015.
260 _aCham :
_bSpringer International Publishing :
_bImprint: Springer,
_c2015.
300 _aXIV, 146 p. 50 illus., 47 illus. in color.
_bonline resource.
336 _atext
_btxt
_2rdacontent
337 _acomputer
_bc
_2rdamedia
338 _aonline resource
_bcr
_2rdacarrier
490 1 _aAdvances in Anatomy, Embryology and Cell Biology,
_x0301-5556 ;
_v217
505 0 _a1. Introduction -- 2. The neuropathological grading of Huntington disease -- 3. The cerebral cortex in Huntington´s disease -- 4. Degeneration of select motor and limbic nuclei of the thalamus in Huntington’s disease -- 5. Consistent and widespread degeneration of the cerebellum in Huntington’s disease -- 6. Elucidation of the role of the premotor oculomotor brainstem nuclei in the pathogenesis of oculomotor dysfunctions in Huntington’s disease -- 7. Widespread brainstem neurodegeneration in Huntington’s disease -- 8. Intraneuronal transport and defense mechanisms with possible pathogenetic relevance in Huntington’s disease -- 9. The disease protein huntingtin and neuronal protein aggregations in Huntington’s disease -- 10. Pathological nerve cell alterations in Huntington’s disease (HD) and their possible role for the demise of nerve cells -- 11. Conclusions and outlook.
520 _aThis 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.
650 0 _amedicine.
_9566220
650 0 _aHuman genetics.
_9566248
650 0 _aNeurosciences.
_9302217
650 0 _aPathology.
_9303087
650 1 4 _aBiomedicine.
_9566246
650 2 4 _aNeurosciences.
_9302217
650 2 4 _aHuman Genetics.
_9566249
650 2 4 _aPathology.
_9303087
700 1 _aVonsattel, Jean Paul G.
_eauthor.
_9466734
700 1 _aHeinsen, Helmut.
_eauthor.
_9466735
700 1 _aKorf, Horst-Werner.
_eauthor.
_9466736
710 2 _aSpringerLink (Online service)
_9143950
773 0 _tSpringer eBooks
830 0 _aAdvances in Anatomy, Embryology and Cell Biology,
_9319584
856 4 0 _uhttp://dx.doi.org/10.1007/978-3-319-19285-7
912 _aZDB-2-SBL
999 _c414732