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Neuropathology and Genetics of Dementia : Critical Issues in Social Justice - Markus Tolnay

Neuropathology and Genetics of Dementia

Critical Issues in Social Justice

By: Markus Tolnay (Editor), Alphonse Probst (Editor)

Hardcover Published: 31st May 2001
ISBN: 9780306465581
Number Of Pages: 255

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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.

Can the Physician Predict the Neuropathologist?p. 1
The Molecular Parameters of Tau Pathology: Tau as a killer and a witnessp. 5
Tau Gene Mutations and Tau Pathology in Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17p. 21
Argyrophilic Grain Disease: A frequent dementing disorder in aged patientsp. 39
Tau Pathology in Neurons and Glial Cells of Aged Baboonsp. 59
Human Tau Transgenic Mice: Towards an animal model for neuro- and glialfibrillary lesion formationp. 71
Regulation of Alzheimer [beta]-Amyloid Precursor Trafficking and Metabolismp. 85
Role of Type 10 17[beta]-Hydroxysteroid Dehydrogenase in the Pathogenesis of Alzheimer's Diseasep. 101
The Spectrum of Vascular Disease in Dementia: From ischemia to amyloid angiopathyp. 111
Transgenic Mouse Models of Cerebral Amyloid Angiopathyp. 123
Alpha-Synuclein: Axonal transport, ligand interaction and neurodegenerationp. 129
Dementia with Lewy Bodiesp. 135
Mouse Models of [alpha]-Synucleinopathy and Lewy Pathology: [alpha]-synuclein expression in transgenic micep. 147
Ubiquitin and the Molecular Pathology of Neurodegenerative Diseasesp. 169
Perisomatic Granules of Hippocampal Ca1 Neurons in Alzheimer's Disease, Pre-Alzheimer Stage and Pick's Disease: an Overlooked Pathological Entityp. 187
Pathological Mechanisms in Polyglutamine Expansion Diseasesp. 199
Dementia in the Neuronal Ceroid-Lipofuscinosesp. 211
Neurodegeneration-Associated Proteins and Inflammation in Sporadic Inclusion-Body Myositisp. 219
Mitochondrial Dysfunction in Neurodegenerative Disorders and Ageingp. 229
Indexp. 253
Table of Contents provided by Syndetics. All Rights Reserved.

ISBN: 9780306465581
ISBN-10: 0306465582
Series: Critical Issues in Social Justice
Audience: General
Format: Hardcover
Language: English
Number Of Pages: 255
Published: 31st May 2001
Publisher: Springer Science+Business Media
Country of Publication: US
Dimensions (cm): 25.4 x 17.78  x 1.6
Weight (kg): 0.69