Book contents
- Frontmatter
- Contents
- List of contributors
- List of abbreviations
- Preface to second edition
- 1 Definition, clinical features and neuroanatomical basis of dementia
- 2 Important anatomical landmarks in the brain in dementia
- 3 Practical approach to pathological diagnosis
- 4 Morphometric methods and dementia
- 5 Safety precautions in laboratories involved with dementia diagnosis and research
- 6 Molecular diagnosis of dementia
- 7 Neuropathology of the ageing brain
- 8 Neuroimaging Alzheimer's disease
- 9 Alzheimer's disease
- 10 Down's syndrome and Alzheimer's disease
- 11 Sporadic tauopathies: Pick's disease, corticobasal degeneration, progressive supranuclear palsy and argyrophilic grain disease
- 12 Hereditary tauopathies and idiopathic frontotemporal dementias
- 13 Vascular dementias
- 14 Familial and sporadic cerebral amyloid angiopathies associated with dementia and the BRI dementias
- 15 Parkinson's disease, dementia with Lewy bodies, multiple system atrophy and the spectrum of diseases with α-synuclein inclusions
- 16 Huntington's disease
- 17 Human prion diseases
- 18 Alcoholism and dementia
- 19 Hydrocephalus and dementia
- 20 Head injury and dementia
- 21 Infectious (and inflammatory) diseases causing dementia
- 22 Schizophrenia and its dementia
- 23 Other diseases that cause dementia
- 24 Transgenic mouse models of neurodegenerative disease
- Appendix: Dementia brain banks
- Index
15 - Parkinson's disease, dementia with Lewy bodies, multiple system atrophy and the spectrum of diseases with α-synuclein inclusions
Published online by Cambridge University Press: 12 October 2009
- Frontmatter
- Contents
- List of contributors
- List of abbreviations
- Preface to second edition
- 1 Definition, clinical features and neuroanatomical basis of dementia
- 2 Important anatomical landmarks in the brain in dementia
- 3 Practical approach to pathological diagnosis
- 4 Morphometric methods and dementia
- 5 Safety precautions in laboratories involved with dementia diagnosis and research
- 6 Molecular diagnosis of dementia
- 7 Neuropathology of the ageing brain
- 8 Neuroimaging Alzheimer's disease
- 9 Alzheimer's disease
- 10 Down's syndrome and Alzheimer's disease
- 11 Sporadic tauopathies: Pick's disease, corticobasal degeneration, progressive supranuclear palsy and argyrophilic grain disease
- 12 Hereditary tauopathies and idiopathic frontotemporal dementias
- 13 Vascular dementias
- 14 Familial and sporadic cerebral amyloid angiopathies associated with dementia and the BRI dementias
- 15 Parkinson's disease, dementia with Lewy bodies, multiple system atrophy and the spectrum of diseases with α-synuclein inclusions
- 16 Huntington's disease
- 17 Human prion diseases
- 18 Alcoholism and dementia
- 19 Hydrocephalus and dementia
- 20 Head injury and dementia
- 21 Infectious (and inflammatory) diseases causing dementia
- 22 Schizophrenia and its dementia
- 23 Other diseases that cause dementia
- 24 Transgenic mouse models of neurodegenerative disease
- Appendix: Dementia brain banks
- Index
Summary
Introduction
Synucleinopathies are a group of neurodegenerative disorders sharing in common the presence of intracellular fibrillar inclusions composed of polymerized α-synuclein (α-syn). Although the biological function of this presynaptic protein remains largely unknown, overwhelming evidence indicates that its self-aggregation in neurons and/or oligodendrocytes is associated with the impairment of nervous system function and cellular demise leading to disease. Pathological inclusions composed of α-syn have been the focus of enumerable clinical and pathological studies with the earliest dating almost to the beginning of the twentieth century. However, the finding that these inclusions are comprised of α-syn only became apparent in the past 5 years following the identification of a mutation in the α-syn gene in families with Parkinson's disease. α-Syn inclusions are the defining characteristics of several disorders including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). However, they are also found in a significant percentage of other neurodegenerative disorders including neurodegeneration with brain iron accumulation type-1 (NBIA-1), Down's syndrome, and familial and sporadic Alzheimer's disease (AD) (Table 15.1). The understanding of the role of α-syn in neurodegenerative disease requires the determination of the normal function and properties of this protein, the clinicopathological assessment of the relationship between α-syn inclusions and disease, and the elucidation of the mechanism of pathogenesis. In this chapter, we will focus on the most recent developments in these aspects of synucleinopathies, but the description of these diseases also requires an overview of the most significant features that have been documented for over almost 100 years.
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- The Neuropathology of Dementia , pp. 353 - 375Publisher: Cambridge University PressPrint publication year: 2004
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