" /> Dementia, lewy body - CISMeF





Preferred Label : Dementia, lewy body;

Symbol : DLB;

CISMeF acronym : DLB;

Type : Phenotype, molecular basis known;

Alternative titles and symbols : Lewy body dementia; Diffuse lewy body disease;

Included titles and symbols : Diffuse lewy body disease with gaze palsy; Lewy body variant of alzheimer disease;

Description : Dementia with Lewy bodies (DLB) is a neurodegenerative disorder clinically characterized by dementia and parkinsonism, often with fluctuating cognitive function, visual hallucinations, falls, syncopal episodes, and sensitivity to neuroleptic medication. Pathologically, Lewy bodies are present in a pattern more widespread than usually observed in Parkinson disease (see PD; 168600). Alzheimer disease (AD; 104300)-associated pathology and spongiform changes may also be seen (McKeith et al., 1996; Mizutani, 2000; McKeith et al., 2005).;

Inheritance : Autosomal dominant;

Molecular basis : Caused by mutation in the alpha-synuclein gene (SNCA, 163890.0004); Caused by mutation in the beta-synuclein gene (SNCB, 602569.0001);

Prefixed ID : #127750;

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02/06/2024


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