ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Alzheimer's disease is a neurodegenerative disorder characterized by the massive and invariant accumulation of amyloid plaques in the brains of affected patients. In many cases Alzheimer's disease occurs in the absence of a prior history of the disease in other family members and is designated as sporadic, whereas in approximately 10% of patients, dominantly transmitted mutations within one of three genes are found. A few mutations have been identified within the gene encoding the β-amyloid precursor protein; however, these mutations account for only about 1–3% of cases with familial Alzheimer's disease. In the majority of autosomal dominant cases (40–50%), mutations have been found in a gene localized to chromosome 14. The responsible gene, now called presenilin-1, has recently been identified and shown to encode a putative seven transmembrane domain protein. Surprisingly, a second highly homologous gene (named presenilin-2) was cloned shortly thereafter. It is localized on human chromosome 1 and is also involved in a small number of cases with familial Alzheimer's disease. Early data suggest that mutations found within the two genes cause early onset Alzheimer's disease by influencing the proteolytic processing of amyloid β-peptide in a pathological manner.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Rapid advances are being made in our understanding of the pathogenesis of neurodegenerative diseases, particularly those in which specific DNA mutations have been identified. β-amyloid has been shown to induce free radical formation both directly and via an effect on endothelial function. There is persuasive evidence for cytochrome oxidase dysfunction with oxidative stress and damage in the brains of patients with Alzheimer's disease. The confirmation of the complex II inhibitor 3-nitropropionic acid as a toxin model for Huntington's disease, together with the demonstration of reduced mitochondrial function in Huntington's disease caudate, supports the proposition that mutant huntingtin may exert its effect through an abnormality of energy metabolism.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Apolipoprotein E became relevant for neurologists in 1993 when the association of the apolipoprotein E-ε4 allele with familial and sporadic late-onset Alzheimer disease was reported. Since that time, more than 100 confirmations and many research papers have appeared. A large neurobiological literature concerning the role of apolipoprotein E in the metabolism of the central nervous system is developing.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Dementia with Lewy bodies is a generic term which was proposed at the first International Workshop on Lewy Body Dementia (Newcastle upon Tyne, 1095). It is an all encompassing term that includes various types of disorder such as diffuse Lewy body disease, senile dementia of Lewy body type, and Lewy body variant of Alzheimer's disease. Epidemiological, clinical, neuropathological, biochemical, molecular biological, and therapeutic contributions to the understanding of dementia with Lewy bodies are reviewed.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Asymmetric cortical degeneration syndromes (aphasic, perceptual-motor, frontal lobe and bitemporal categories) encompass the majority of atypical cortical dementia patterns. New insights suggest the group is genetically heterogeneous. Debate continues over whether individual syndromes are points along a continuum or distinct nosologic entities.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

We have summarized relevant literature on the patterns of memory deficit found in Alzheimer's disease, the focal lobar atrophies (semantic dementia and dementia of frontal type) and three forms of subcortical dementia (progressive supranuclear palsy, Huntington's and Parkinson's disease). We have stressed an approach based on the joint principals of cognitive neuropsychology and applied neuroanatomy. Alzheimer's disease results in a severe deficit in the establishment of new episodic memories which reflects the perihippocampal locus of the early pathology. In semantic dementia, there is profound progressive and relatively pure loss of semantic memory associated with focal left temporal neocortical atrophy. By contrast, in the subcortical dementias the major impairments are in working (immediate) memory and retrieval of information from longer term memory stores, deficits which reflect dysfunction of frontostriatal systems.

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID

ISSN:1350-7540    

出版商:OVID    

年代:1996

数据来源: OVID