Year: 2026
Type: article
Abstract: Essential tremor (ET) is a neurodegenerative motor disease [1][2][3][4] whose defining clinical feature is kinetic tremor [5].ET patients also have been shown to perform more poorly on cognitive testing than age-matched controls and furthermore, epidemiological studies have demonstrated that a diagnosis of ET significantly increases risk for dementia (relative risks = 1.64-1.89)[6,7].In fact, longitudinal data indicate that conversion rate from normal cognition to dementia in older adults with ET is approximately three-times higher than in older adults without ET [8].Hence, we are witnessing the emerging recognition of a new association -ET with dementia.The pathogenesis of this dementia is not clear, however initial studies suggest that there is a predisposition for ET patients to develop Alzheimer's disease (AD) [9,10].Although scientifically intriguing, given the very high prevalence of ET (2.2% of the entire US population -7 million people) [11], the increased risk of dementia is troubling from both clinical and public health perspectives.The association between ET and dementia has been studied using case-control and cohort studies, assessing the relative prevalence and/or incidence of dementia in ET versus control groups.Another pathway to address whether dementia is disease-associated in ET is to conduct a familial aggregation study.Familial aggregation studies ask whether relatives of ET cases have cognitive impairment rather than whether ET cases themselves have cognitive impairment, and whether this level of cognitive impairment exceeds that seen in relatives of controls.In the absence of a control group, the prevalence of dementia in ET relatives may be compared with the prevalence of dementia in relatives of historical controls.Here, we report the results of such analyses.ET cases were enrolled in a prospective, longitudinal, nationwide study of older adults with ET (Clinical Pathological Study of Cognitive Impairment in Essential Tremor [COGNET]; National Institutes of Health Award #R01 NS086736), described in detail [8].To date, cases reside in 43 US states, having responded to an advertisement to enroll in this cohort [8].The Yale University, Columbia University, and University of Texas Southwestern Medical Center Institutional Review Boards approved the study protocol.All cases provided written, informed consent.During the in-home baseline evaluation, ET cases were asked "Do you have a family history of dementia?"Data were available on 132 ET cases whose mean age was 76.5 years (range = 65-97 years).Of these, 55 (41.7%, 95% confidence interval = 33.2% to 50.6%) reported that a relative had dementia.Among these 132 were 43 (32.6%) who reported a first-degree relative with dementia and 15 (11.4%) who reported a second-degree relative with dementia.Among the 55 who
DOI: 10.5334/tohm.1289
Authors: Elan D. Louis, Stephanie Cosentino
Language: English
Subfield: Psychiatry and Mental health
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $800
Funder: National Institutes of Health
OpenAlex ID: W7223173390