Rare genetically defined causes of dementia

Several genetic disorders, though rare, are associated or present with dementia. Developments in the field of genetics are contributing to clarify and expand our knowledge of the complex physiopathological mechanisms leading to neurodegeneration and cognitive decline. Disorders associated with misfo...

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Bibliographic Details
Published in:International psychogeriatrics 2005-09, Vol.17 (s1), p.S149-S194
Main Authors: Novakovic, K. E., Villemagne, V. L., Rowe, C. C., Masters, C. L.
Format: Article
Language:English
Subjects:
Alzheimer's disease
Amyotrophic Lateral Sclerosis - complications
Dementia
Dementia - complications
Dementia - diagnosis
Dementia - genetics
Diagnosis, Differential
Energy Metabolism - physiology
Gerstmann-Straussler-Scheinker Disease - genetics
Humans
Huntington Disease - complications
Insomnia, Fatal Familial - complications
Insomnia, Fatal Familial - genetics
Lipoproteins - metabolism
Nerve Degeneration - complications
Parkinson's disease
Proteins - metabolism
Spinocerebellar Ataxias - complications
Supranuclear Palsy, Progressive - complications
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Summary:Several genetic disorders, though rare, are associated or present with dementia. Developments in the field of genetics are contributing to clarify and expand our knowledge of the complex physiopathological mechanisms leading to neurodegeneration and cognitive decline. Disorders associated with misfolded and aggregated proteins and lipid, metal or energy metabolism are examples of the multifarious disease processes converging in the clinical features of dementia, either as its predominant feature, as in cases of Alzheimer's disease (AD) or frontotemporal dementia (FTD), or as part of a cohort of accompanying or late-developing symptoms, as in Parkinson's disease (PD) or amyotrophic lateral sclerosis with dementia (ALS-D). Awareness of these disorders, allied with recent advances in genetic, biochemical and neuroimaging techniques, may lead to early diagnosis, successful treatment and better prognosis.
ISSN:1041-6102
1741-203X
DOI:10.1017/S1041610205002012