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
12 - Hereditary tauopathies and idiopathic frontotemporal dementias
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
List of abbreviations
Aβ, β-amyloid; AD, Alzheimer's disease; ALS, amyotrophic lateral sclerosis; CBD, corticobasal degeneration; CHO, Chinese hamster ovary; CNS, central nervous system; ESE, exon-splicing enhancer; ESS, exon-splicing silencer; FTD, frontotemporal dementia; FTDP-17, frontotemporal dementia with parkinsonism linked to chromosome 17; FTLD, frontotemporal lobar degeneration; IHC, immunohistochemistry; MAP, microtubule-associated protein; MND, motor neuron disease; MT, microtubule; NFT, neurofibrillary tangle; PD, Parkinson's disease; PDC, parkinsonism-dementia complex; PHF, paired helical filament; PiD, Pick's disease; PSG, progressive subcortical gliosis; PSP, progressive supranuclear palsy; SF, straight filament; TG, transgenic; WT, wild-type.
Introduction
In 1892, Arnold Pick described a woman with lobar brain atrophy who presented clinically with presenile dementia and aphasia (Pick, 1892), and thus, the first description of what is now classified clinically as frontotemporal dementia (FTD) (Neary et al., 1998; McKhann et al., 2001). The clinical syndromes of FTD are associated with several neuropathological abnormalities (Lund and Manchester Groups, 1994; McKhann et al., 2001). A subset of these disorders is characterized by the intracellular accumulations of filamentous material composed of the microtubule-associated protein (MAP) tau. The term ‘tauopathies’ was coined to refer to this seemingly heterogeneous group of neurodegenerative disorders that includes Pick's disease (PiD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), neurofibrillary tangle dementia, and argyrophilic grain disease (see ‘Sporadic tauopathies’, Chapter 11). In FTD patients, there is a family history of a similar dementing illness in approximately 38 to 50% of patients (Knopman et al., 1990b; Stevens et al., 1998; Chow et al., 1999).
- Type
- Chapter
- Information
- The Neuropathology of Dementia , pp. 257 - 288Publisher: Cambridge University PressPrint publication year: 2004
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