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.
