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.
