Abstract：
:This chapter reviews the neurological phenotype of Down syndrome (DS) in early development, childhood, and aging. Neuroanatomic abnormalities in DS are manifested as aberrations in gross brain structure as well as characteristic microdysgenetic changes. As the result of these morphological abnormalities, brain circuitry is impaired. While an intellectual disability is ubiquitous in DS, there is a wide range of variation in cognitive performance and a growing understanding between aberrant brain circuitry and the cognitive phenotype. Hypotonia is most marked at birth, affecting gait and ligamentous laxity. Seizures are bimodal in presentation with infantile spasms common in infancy and generalized seizures associated with cognitive decline observed in later years. While all individuals have the characteristic neuropathology of Alzheimer's disease (AD) by age 40 years, the prevalence of dementia is not universal. The tendency to develop AD is related, in part, to several genes on chromosome 21 that are overexpressed in DS. Intraneuronal accumulation of β-amyloid appears to trigger a cascade of neurodegeneration resulting in the neuropathological and clinical manifestations of dementia. Functional brain imaging has elucidated the temporal sequence of amyloid deposition and glucose metabolic rate in the development of dementia in DS. Mitochondrial abnormalities contribute to oxidative stress which is part of AD pathogenesis in DS as well as AD in the general population. A variety of medical comorbidities threaten cognitive performance including sleep apnea, abnormalities in thyroid metabolism, and behavioral disturbances. Mouse models for DS are providing a platform for the formulation of clinical trials with intervention targeted to synaptic plasticity, brain biochemistry, and morphological brain alterations.
journal_name
Prog Brain Resjournal_title
Progress in brain researchauthors
Lott ITdoi
10.1016/B978-0-444-54299-1.00006-6subject
Has Abstractpub_date
2012-01-01 00:00:00pages
101-21eissn
0079-6123issn
1875-7855pii
B978-0-444-54299-1.00006-6journal_volume
197pub_type
杂志文章,评审abstract：:Impulsive testing, which accurately measures the gain of the vestibulo-ocular reflex (VOR) is mostly recorded from only one eye. We measured the horizontal VOR of both eyes in response to graded velocity head impulses in 14 normal subjects. VOR acceleration gains were similar for each eye in both directions at low acc...
journal_title：Progress in brain research
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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journal_title：Progress in brain research
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更新日期：2002-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2010-01-01 00:00:00
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更新日期：2006-01-01 00:00:00
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更新日期：2004-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2019-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2011-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2018-01-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2009-01-01 00:00:00
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pub_type： 杂志文章
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更新日期：2013-01-01 00:00:00
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journal_title：Progress in brain research
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更新日期：2017-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2011-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2009-01-01 00:00:00
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journal_title：Progress in brain research
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journal_title：Progress in brain research
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pub_type： 杂志文章,评审
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更新日期：2008-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章
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更新日期：1993-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2008-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
doi：10.1016/B978-0-444-53839-0.00019-3
更新日期：2011-01-01 00:00:00
abstract：:By blocking electrical activity in the cerebellar cortex the Purkinje cell dendrites become a uniform territory with a high density of spines all bearing the glutamate receptor delta2 subunit (GluRdelta2) and being mainly innervated by parallel fibers. Such a subunit, which is constitutively targeted specifically to t...
journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2005-01-01 00:00:00
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journal_title：Progress in brain research
pub_type： 杂志文章,评审
doi：10.1016/S0079-6123(03)43025-2
更新日期：2004-01-01 00:00:00
abstract：:Language is predominantly a left hemisphere function, yet patients with extensive damage to known language areas often recover quite well in the days to weeks to even years following focal brain injury. This recovery period can be divided into three overlapping stages: acute, subacute, and chronic, each with different...
journal_title：Progress in brain research
pub_type： 杂志文章,评审
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更新日期：2006-01-01 00:00:00
abstract：:This chapter describes current findings from the research into postoperative cognitive dysfunction (POCD) following cardiac and non-cardiac surgery in older adults. The evidence suggests that a significant proportion of patients show POCD in the early weeks following surgery and anesthesia. Specific domains of cogniti...
journal_title：Progress in brain research
pub_type： 杂志文章,评审
doi：10.1016/S0079-6123(07)00026-X
更新日期：2008-01-01 00:00:00
abstract：:The aim of this chapter is to review recent literature describing how developments in cognition may contribute to age-related changes in emotional processes, specifically emotion regulation and emotion perception. In general, older adults are more likely than young adults to report feeling positive. Prominent conceptu...
journal_title：Progress in brain research
pub_type： 杂志文章,评审
doi：10.1016/bs.pbr.2019.02.007
更新日期：2019-01-01 00:00:00
