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
10 - Down's syndrome and Alzheimer's disease
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
Down's syndrome (DS) occurs in about 1/800 live births (Adams et al., 1981) and accounts for about 17% of the mentally handicapped population (Heller, 1969). An association between DS and dementia was first noted well over a century ago by Fraser & Mitchell (1876), who wrote ‘in not a few instances, however, death was attributed to nothing more than a general decay – a sort of precipitated senility’. Nevertheless, it was not until much later (Struwe, 1929; Jervis, 1948) that the linkage between this ‘senile decay’ and the occurrence within the brain of the pathological lesions of Alzheimer's disease (AD), namely senile plaques (SP) and neurofibrillary tangles (NFT), was noted. More recently, a number of studies have shown there to be an excess of DS births among the relatives of AD patients, particularly early onset AD families (Heston et al., 1981; Heyman et al., 1983; Broe et al., 1990; Van Duijn et al., 1991). Conversely, there is an increased risk of AD among mothers of DS children (Yatham et al., 1988; Schupf et al., 1994). Such observations support the pathological observations of shared etiopathogenetic causes for AD and DS. Life expectancy for people with DS has progressively risen with nearly half individuals living to beyond 50 years of age (Dupont et al., 1986; Baird & Sadovnick, 1987; Holland & Moss 1997) and with this the problems of ‘precocious ageing’ and dementia in DS have gained prominence.
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- The Neuropathology of Dementia , pp. 207 - 226Publisher: Cambridge University PressPrint publication year: 2004