Family History is Associated with Phenotype in Dementia with Lewy Bodies.
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ABSTRACT:
SUBMITTER: Vergouw LJM
PROVIDER: S-EPMC7029358 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
Publications
Family History is Associated with Phenotype in Dementia with Lewy Bodies.
Vergouw Leonie J M LJM Bosman Brechje B van de Beek Marleen M Salomé Mariet M Hoogers Susanne E SE van Steenoven Inger I Roks Gerwin G Bonifati Vincenzo V van Swieten John C JC Lemstra Afina W AW de Jong Frank Jan FJ
Journal of Alzheimer's disease : JAD 20200101 1
It is currently unknown whether patients with dementia with Lewy bodies (DLB) with relatives with dementia or Parkinson's disease (familial DLB patients) have a different phenotype than sporadic DLB patients. In this study, we aimed to examine disease onset, rate of cognitive decline, survival, and Alzheimer's disease (AD) biomarkers in patients with familial DLB (n = 154) and sporadic DLB (n = 137), using linear mixed model analysis and Cox regression analysis, among others. Familial patients h ...[more]
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Project description:Dementia with Lewy bodies (DLB) is a clinical diagnosis representing a specific presentation of a pathological α-synucleinopathy (Lewy body disease). DLB is one entity under the broader term Lewy body dementia, which also includes Parkinson's disease dementia. Recent advances in DLB include publication of updated diagnostic criteria and recognition of prodromal DLB states, including mild cognitive impairment, delirium-onset, and psychiatric-onset forms. Research criteria for the mild cognitive impairment form of DLB were published in 2020. Increasing research shows that concomitant Alzheimer's disease pathology in individuals with DLB is common in addition to the α-synucleinopathy pathology. This has implications for biomarker use and expected progression. Identifying biomarkers for DLB is an area of active research. Cerebrospinal fluid and skin biopsy tests are now commercially available in the United States, but their role in routine clinical care is not yet established. Additional research and biomarkers are needed. Research suggests that median survival after DLB diagnosis is 3-4 years, but there are rapidly and slowly progressive forms. Most individuals with DLB die of complications of the disease. Clinical trials for individuals with DLB have increased over the last 5 years, targeting both symptoms and underlying pathology. Effective therapies remain an unmet need, however. This review focuses on recent advances with an emphasis on literature that informs clinical care.
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