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Neuropathology and Genetics of Dementia (Hardcover, 2001 ed.)
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Neuropathology and Genetics of Dementia (Hardcover, 2001 ed.)
Series: Advances in Experimental Medicine and Biology, 487
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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.
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