000			03820nam a22005295i 4500
001			978-3-319-19285-7
003			DE-He213
005			20160405112907.0
007			cr nn 008mamaa
008			150929s2015 gw | s |||| 0|eng d
020			_a9783319192857 _9978-3-319-19285-7
024	7		_a10.1007/978-3-319-19285-7 _2doi
050		4	_aRC321-580
072		7	_aPSAN _2bicssc
072		7	_aMED057000 _2bisacsh
082	0	4	_a612.8 _223
100	1		_aRüb, Udo. _eauthor.
245	1	4	_aThe Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy _h[electronic resource] / _cby Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf.
250			_a1st ed. 2015.
264		1	_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
347			_atext file _bPDF _2rda
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.
650		0	_aHuman genetics.
650		0	_aNeurosciences.
650		0	_aPathology.
650	1	4	_aBiomedicine.
650	2	4	_aNeurosciences.
650	2	4	_aHuman Genetics.
650	2	4	_aPathology.
700	1		_aVonsattel, Jean Paul G. _eauthor.
700	1		_aHeinsen, Helmut. _eauthor.
700	1		_aKorf, Horst-Werner. _eauthor.
710	2		_aSpringerLink (Online service)
773	0		_tSpringer eBooks
776	0	8	_iPrinted edition: _z9783319192840
830		0	_aAdvances in Anatomy, Embryology and Cell Biology, _x0301-5556 ; _v217
856	4	0	_uhttp://dx.doi.org/10.1007/978-3-319-19285-7
912			_aZDB-2-SBL
999			_c4073 _d4073