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Background Essential tremor (ET), a highly-prevalent, progressive, aging-associated neurological disease, poses a significant public health challenge. Aside from its associated motor features, the disease affects cognition in some patients, mood, and morale, and has been associated with increased risk of mortality in the elderly. Studies have not investigated the relationship between self-rated health and risk of ET. We determined whether baseline poor self-rated health was associated with increased risk of incident ET. Methods In this prospective, population-based study of people ≥65 years of age, subjects were evaluated at baseline and 3 years later. At baseline, subjects were asked to rate their health using a single-item health question. Hazard ratios (HR) of ET according to baseline self-rated health (very good, good, fair, poor, and very poor) were estimated with Cox models. Results The 3853 subjects had a median follow-up duration of 3.2 years. There were 82 incident ET cases. Compared to subjects who rated their health as good or very good, the risk of ET was increased for subjects who rated their health as fair (HR = 1.69, p = 0.03), and for subjects who rated their health as poor or very poor (HR = 2.12, p = 0.02). In a fully adjusted model, the risk remained increased for subjects who rated their health as poor or very poor (HR = 2.34, p = 0.01). Conclusions Subjects rating their health as poor/very poor at baseline were at increased risk of incident ET at follow-up. Premotor and/or early motor symptoms of ET could negatively influence self-perceptions of health status. [ABSTRACT FROM AUTHOR]

Background Studies investigating the association between hypercholesterolemia in the elderly and cognitive decline report discrepant outcomes. We determined in a prospective population-based cohort (NEDICES) in elders whether hypercholesterolemia was associated with slower cognitive decline. Methods Participants were evaluated at baseline and 3 years later. Baseline demographic variables were recorded. Hypercholesterolemia was defined by total cholesterol of > 200 mg/dl or current use of lipid-lowering drugs. At baseline and at follow-up, a 37-item version of the Mini-Mental State Examination (37-MMSE) was administered. Results The final sample, 2015 participants (72.9 ± 6.1 years), comprised 1166 (57.9%) hypercholesterolemic and 849 (42.1%) non-hypercholesterolemic participants (reference category). The mean follow-up was 3.4 ± 0.5 years. During the three year follow-up period, the 37-MMSE declined by 0.7 ± 4.3 points (median = 0 point) in non-hypercholesterolemic participants vs. 0.3 ± 3.9 points in hypercholesterolemic participants (median = 0 points) (Mann–Whitney test, p = 0.007). In analyses adjusted for baseline age and other potential confounders, this difference remained robust. We also assessed the cognitive decline per unit time (i.e., the rate of cognitive decline). The rate of cognitive decline was 0.2 ± 1.3 (median = 0.0) points/year for non-hypercholesterolemic participants and 0.1 ± 1.2 (median = 0.0) points/year for hypercholesterolemic participants (Mann–Whitney test, p = 0.028). Conclusions In this prospective population-based cohort study, cognitive test scores among hypercholesterolemic elders declined more slowly than observed in their non-hypercholesterolemic counterparts. Additional studies are needed to confirm these results. [ABSTRACT FROM AUTHOR]

Background Parkinson's disease is frequently omitted as a cause of death from death certificates. A limitation of previous studies that attempted to assess the validity of death certificates is that population-dwelling cases, with milder, undiagnosed Parkinson's disease were likely excluded. As a result, those studies likely overestimated the validity of death certificates because they did not include these milder cases. We assessed the validity of death certificates in a prospective population-based study (NEDICES), which includes previously undiagnosed Parkinson's disease cases detected during the assessment. Methods 3926 community-dwelling elderly subjects with and without Parkinson's disease were followed during a median of 12.6 years, after which the death certificates of those who died were examined. We calculated the proportion of cases of clinically diagnosed Parkinson's disease for whom a diagnosis of Parkinson's disease was certified as the basic cause of death on death certificates. Results 1791 (45.6%) of the 3926 participants died over a median follow-up of 7.1 years, including 82 (73.9%) deaths among 111 participants with Parkinson's disease. Parkinson's disease was rarely certified as the basic cause of death (14.6%). Gender, disease stage and the period during which the study was conducted (i.e., 1994 to 2007) did not influence the likelihood that Parkinson's disease would be reported. Conclusions Our findings reinforce the notion that the reporting of Parkinson's disease on death certificates remains poor. This suggests a lack of awareness of the importance of Parkinson's disease as a cause of death. [ABSTRACT FROM AUTHOR]

Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is an autosomal recessive vascular disorder caused by biallellic variants in HTRA1. Recently, it has been reported that several heterozygous mutations in HTRA1 are responsible for a milder late-onset cerebral small vessel disease (CSVD) with an autosomal dominant pattern of inheritance. The majority of them are missense that affects the Htr1A protease activity due to a dominant-negative effect caused by defective trimerization or monomer activation. The molecular mechanism related to the structural destabilization of the protein supports the practical utility of integrating computational stability predictors to prioritize candidate variants in this gene. In this work, we report a family with several members diagnosed with subcortical ischemic events and progressive cognitive impairment caused by the novel c.820C > G, p.(Arg274Gly) heterozygous variant in HTRA1 segregating in an autosomal dominant manner and propose its molecular mechanism by a three-dimensional model of the protein's structure. [ABSTRACT FROM AUTHOR]