Relationship of Down’s Syndrome to Alzheimer’s Disease

Rabe, Ausma; Wisniewski, Krystyna E.; Schupf, Nicole; Wı́sniewski, Henryk M.

doi:10.1007/978-1-4613-0525-5_21

Cited by 17 publications

(10 citation statements)

References 111 publications

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“…53 How syndrome-specific is the combination of neuroanatomic abnormalities observed in this sample of DS patients? One of the most striking of these abnormalities is hypoplasia of the cerebellum.…”

Section: Figure 4 Three Sketches Showing the Differences In Regionalmentioning

confidence: 99%

Selective neuroanatornic abnormalities in Down's syndrome and their cognitive correlates

et al. 1995

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We examined the pattern of neuroanatomic abnormalities in adults with Down's syndrome (DS) and the cognitive correlates of these abnormalities. Specifically, we compared this pattern with what would be predicted by the hypotheses attributing DS pathology to either premature aging or Alzheimer's disease. We measured a number of brain regions on MRIs of 25 subjects: 13 persons with the DS phenotype and 12 age- and sex-matched healthy volunteers. Study participants had no history of cardiovascular disease, diabetes, thyroid dysfunction, or seizure disorder. After statistical adjustment for differences in body size, we found that, in comparison with controls, DS subjects had substantially smaller cerebral and cerebellar hemispheres, ventral pons, mammillary bodies, and hippocampal formations. In the cerebellar vermis of DS subjects, we observed smaller lobules VI to VIII without appreciable differences in other regions. In addition, we noted trends for shrinkage of the dorsolateral prefrontal cortex, anterior cingulate gyrus, inferior temporal and parietal cortices, parietal white matter, and pericalcarine cortex in DS subjects compared with normal controls. The parahippocampal gyrus was larger in DS subjects. We found no significant group differences in the volumes of the prefrontal white matter, the orbitofrontal cortex, the pre- and postcentral gyri, or the basal ganglia. We conclude that the pattern of selective cerebral damage in DS does not clearly fit the predictions of the premature aging or Alzheimer's disease hypotheses. To examine the relationship between brain abnormalities and cognitive deficits observed in DS, we correlated the size of brain regions that were significantly reduced in DS with performance on tests of intelligence and language. The correlation analysis suggested age-related decline in the DS subjects in general intelligence and basic linguistic skills. General intelligence and mastery of linguistic concepts correlated negatively with the volume of the parahippocampal gyrus. There was no relationship between total brain size and the cognitive variables.

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Section: Figure 4 Three Sketches Showing the Differences In Regionalmentioning

confidence: 99%

Selective neuroanatornic abnormalities in Down's syndrome and their cognitive correlates

et al. 1995

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“…The IQ of people with Down's syndrome is usually in the mild to moderately severe range of the learning disability spectrum (IQ < 70). The prevalence of Alzheimer's disease in people with Down's syndrome rises with increasing age, and prevalence rates varying from 6% to 75% have been reported (Rabe et al, 1990;Visser et al, 1997). A recent study of 285 people with Down's syndrome found a 13% prevalence of Alzheimer's disease (Tyrell et al, 2001).…”

Section: Epidemiology and Aetiologymentioning

confidence: 99%

Down's syndrome and dementia

Stanton¹,

Coetzee²

2004

Adv. psychiatr. treat

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). Her research interests are in liaison in old age psychiatry, the interface between old age psychiatry and learning disability services, and neuropsychiatry. Rikus Coetzee is also a specialist registrar in general adult psychiatry and old age psychiatry at Duncan Macmillan House, Nottingham. His research interests are in the treatment and epidemiology of trauma-related neurosis in the elderly and the use of cognitive-behavioural therapy with elderly people. Down's syndrome and dementiaLisa R. Stanton & Rikus H. Coetzee Abstract Down's syndrome is the most common genetic disorder seen in clinical practice: about 94% occurs because of non-disjunction of chromosome 21 and 3-5% because of translocation. Individuals increasingly survive to middle and old age, probably because of advances in medical treatment and improved living conditions. People with Down's syndrome have an increased risk of developing Alzheimer's disease in middle age. Within ICD-10 and DSM-IV classifications there is no consensus on the diagnosis of dementia in people with learning disability. New treatments have been licensed for use in mild to moderate Alzheimer's disease (e.g. acetylcholinesterase inhibitors and memantine). The comorbid picture of Down's syndrome and Alzheimer's disease presents a unique challenge to the clinician in both diagnosis and management.

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“…It could act in glia to alter calcium-dependent processes or it could also be released and stimulate neurons directly to increase the free intracellular Ca 2+ levels (Barger and Van Eldik 1992). Furthermore, we decided to overexpress the gene for S100~3 instead of eliminating expression because elevated levels of S10013 have been implicated in human neurodegenerative diseases, including Down's syndrome and Alzheimer disease (Rabe et al 1990;Griffin et al 1989).…”

Section: Abnormal Learning and Ltd In 5100~ Transgenic Micementioning

confidence: 99%

“…Overexpression of one or more of these genes could lead to mental retardation (Rabe et al 1990). It is noteworthy that S10013 has also been shown to be expressed in elevated amounts in Alzheimer disease (Griffin et al 1989), and this expression could conceivably influence higher brain functions.…”

Section: Overexpression Of Siooi3 Impairs But Does Not Abolish Spatiamentioning

confidence: 99%

Overexpression of a calcium-binding protein, S100 beta, in astrocytes alters synaptic plasticity and impairs spatial learning in transgenic mice.

Gerlai

Wojtowicz²,

Marks³

et al. 1995

Learn. Mem.

129

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Relationship of Down’s Syndrome to Alzheimer’s Disease

Cited by 17 publications

References 111 publications

Selective neuroanatornic abnormalities in Down's syndrome and their cognitive correlates

Selective neuroanatornic abnormalities in Down's syndrome and their cognitive correlates

Down's syndrome and dementia

Overexpression of a calcium-binding protein, S100 beta, in astrocytes alters synaptic plasticity and impairs spatial learning in transgenic mice.

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