Neuropathology and Genetics of Dementia

For more than 35 years the Swiss Society of Neuropathology has organiseditstraditionalInternationalWinterMeeting.Themainaimofthis venue is to bring together neuropathologists and clinicians as well as neuroscientistsinterestedindiseasemechanisms.TheXVIIIthInternational WinterMeeting washeld inMarch 2000 in St. Moritz, Switzerland. The topicofthe2000Meetingwas"NeuropathologyandGeneticsofDementia". Aprogrammeofinvitedplenarylecturesofhigheducationalvalueaswellas platform andposterpresentations given by manyparticipantscoveredthe broadspectrumofdementingdisorders.Encouragedbythehighstandardof the meeting, and probably also influenced by the advent of a new Millenniumwedecided,togetherwithKluwerAcademiclPlenumPublishers, topublishtheProceedingsofthe2000Meetinginthepresentbook. The increaseofthe agedpopulation challenges social and health care systemsinindustrializedsocieties.Maintenanceofhealthandautonomyinto oldage isakeyfactor,andtheautonomyandindependenceofoldpersons crucially depend on mental health. However, neurodegenerative disorders accompanied bydementiaaffect 5-10%ofindividualsoverthe ageof65, and for the age group between 65 and 95 the prevalence of cognitive dysfunction doubles every 5 years. With this challenge in mind, Rossor discusses the clinical syndrome of dementia which encompasses an enormousvarietyofunderlyingdisorders. Over the last few years it has become apparent that many neurodegenerative disorders are characterised by protein misfolding and aberrant polymerization and may therefore be viewed as "aggregation proteopathies". Among them a substantial subsetofdementing disorders, either with or without motor dysfunction, are characterised by abundant v VI Preface neuronaland/orglialaccumulationofhyperphosphorylatedtauprotein.The molecular parametersofthese "tauopathies" are reviewed by Delacourte. Therecentdiscoverythatmutationsofthetaugenearecausativeforfamilial formsoffrontotemporaldementiaandParkinsonism(FTDP-17)broughtthe "taupathway"and the"tauopathies"tothecentrestage.Thisextraordinary breakthroughwillbecoveredbySpillantiniandGoedert.Thereisincreasing evidence that argyrophilic grain disease is much more frequent in older patients than previously thought. Tolnay et al. are reviewing recent contributionstothepathologyofthislate-onsetdementingdisorderinwhich ofamyloidbetadeposits,isalmost taupathology,inthealmosttotalabsence exclusivelyrestricted to the limbic lobe and the amygdala. Schultzet al. report an intriguing age-related progression of neuronal and glial tau pathologyinbaboonsthusrenderingthe baboonapotentialprimatemodel forage-relatedhuman"tauopathies". Gotzetal. introducetransgenicmice expressingmutated human tau as valuable animal models for reproducing formationofneuro-andglialfibrillarylesions.

Klappentext

This volume is the proceedings from the Swiss Society for Neuropathology XVIIIth International Winter Meeting on Neuropathology and Genetics of Dementia, held March 23-26, 2000, in St. Moritz, Switzerland. For more than 35 years the Swiss Society of Neuropathology has organised its traditional International Winter Meeting, whose main aim is to bring together neuropathologists and clinicians as well as neuroscientists interested in disease mechanisms. The topic of the 2000 Meeting was Neuropathology and Genetics of Dementia. A programme of invited plenary lectures of high educational value as well as platform and poster presentations given by many participants covered the broad spectrum of dementing disorders. Encouraged by the high standard of the meeting, and probably also influenced by the advent of a new Millennium, it was decided to publish the Proceedings of the 2000 Meeting in the present book.

Inhalt
Can the Physician Predict the Neuropathologist?.- The Molecular Parameters Of Tau Pathology.- Tau as a killer and a witness.- Tau Gene Mutations and Tau Pathology in Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17.- Argyrophilic Grain Disease.- A frequent dementing disorder in aged patients.- Tau Pathology in Neurons and Glial Cells of Aged Baboons.- Human Tau Transgenic Mice.- Towards an animal model for neuro-and glialfibrillary lesion formation.- Regulation of Alzheimer 13-Amyloid Precursor Trafficking and Metabolism.- Role of Type 10 1713-Hydroxysteroid Dehydrogenase in the Pathogenesis of Alzheimer's Disease.- The Spectrum Of Vascular Disease In Dementia.- From ischemia to amyloid angiopathy.- Transgenic Mouse Models of Cerebral Amyloid Angiopathy.- Alpha-Synuclein.- Axonal transport, ligand interaction and neurodegeneration.- Dementia with Lewy Bodies.- Mouse Models of ?-Synucleinopathy and Lewy Pathology.- ?-synuclein expression in transgenic mice.- Ubiquitin and the Molecular Pathology of Neurodegenerative Diseases.- Perisomatic Granules of Hippocampal CA1 Neurons in Alzheimer's Disease, Pre-Alzheimer Stage and Pick's Disease: an Overlooked Pathological Entity.- Ubiquitin and the Molecular Pathology of Neurodegenerative Diseasespathological Mechanisms in Polyglutamine Expansion Diseases.- Dementia in the Neuronal Ceroid-Lipofuscinoses.- Neurodegeneration-Associated Proteins and Inflammation in Sporadic Inclusion-Body Myositis.- Mitochondrial Dysfunction in Neurodegenerative Disorders and Ageing.