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Metabolic dementia 

Metabolic dementia
Chapter:
Metabolic dementia
Author(s):

Nicholas J.C. Smith

and Timothy M. Cox

DOI:
10.1093/med/9780199655946.003.0024
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date: 28 May 2020

Metabolic causes of neurocognitive impairment occur in numerous diseases. These may be acquired, arise from single-gene defects (inborn errors of metabolism), or represent complex outcomes from adverse gene–environment interactions. While they are much less frequent than primary neurodegenerative disorders, metabolic diseases with early-onset dementia, particularly where there are multiple neurological manifestations (‘dementia plus’), or pathological effects beyond the nervous system place a heavy burden of disability and present many challenges for management. Prompt recognition of metabolic dementia is essential: specific treatments are often available and almost invariably because there are immediate genetic implications for family members. Here we set out a general framework for the clinical assessment and investigation of cognitive decline where a metabolic cause is, or should be suspected. It provides a conspectus of the diseases which may be encountered, with representative examples. Additional online information accompanies this chapter and supplies references to a detailed specialist bibliography.

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