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Background: Neuropathic tremor occurs with damage to the peripheral nervous system. Guillain-Barré syndrome (GBS) causes acute paralysis following nerve inflammation sometimes resulting in long-term disability. It is unclear how frequent and severe tremor is following GBS., Objectives: We aimed to assess the frequency and features of tremor following GBS., Methods: We enrolled 18 patients with GBS treated in a secondary care center within a 4-year period. Evaluations were done with the Fahn-Tolosa-Marin tremor rating scale (FTM-TRS). We compared these features with a cohort of consecutive patients with untreated essential tremor (ET)., Results: There were 13 males and 5 females with a mean age at evaluation (S.D.) of 41.5 ± 14.0 years and at GBS onset of 40.2 ± 13.7. No patient had history of tremor before GBS. Upper limb tremor was identified in 16 (89%) cases, 35.5% of patients had FTM-TRS score ≥10 points. Tremor was mostly kinetic, jerky with low amplitude with a total score of 10.94 ± 11.84 in the FTM-TRS. Compared with patients with ET, those with GBS-tremor were younger and had lower scores in all subscales of the FTM-TRS ( P value < 0.05 for all comparisons). In a multivariate linear regression analysis "days of hospitalization" had a positive association with the total FTM-TRS score ( P = 0.001)., Conclusions: Tremor was common following GBS. This tremor is mild compared with patients with ET, but adds functional impact., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2024 The Author(s).)

Background: Prior studies suggest that patients with essential tremor (ET) have increased rates of healthcare utilization, but the reason for this increased use is unknown. The objective of this study was to evaluate the reasons for healthcare use among ET patients., Methods: This was a retrospective cross-sectional study of ET patients with an admission or emergency department (ED) visit at a tertiary health system from 2018-2023. Patients were matched on an encounter level with control patients based on propensity scores incorporating age, sex, race, and co-morbid conditions. The primary outcome was the odds of an encounter for each diagnostic category comparing ET patients with matched controls., Results: Only inpatient admissions for neurologic diagnoses were more likely for ET compared to control patients (odds ratio (OR) 3.73, 95% confidence interval (CI) 2.54 - 5.49, p < 0.001). Once admissions related to the surgical treatment of tremor were excluded, admissions for neurologic diagnoses were equally likely among ET and control patients (OR 0.96, 95% CI 0.59 - 1.57, p = 0.88)., Discussion: Surgical treatment of tremor appears to be a key driver of healthcare use among ET patients. Future investigations should examine the pattern of healthcare use of ET patients before and after surgery., Highlights: Prior studies have shown increased healthcare use among essential tremor (ET) patients. The objective of this study was to evaluate the reasons for healthcare use among ET patients compared to matched control patients. Surgical treatment of tremor was found to be a key driver of healthcare use among ET patients., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2024 The Author(s).)

Objective: Despite recent attention to cognitive impairment in essential tremor, few studies examine rates of conversion to diagnoses of mild cognitive impairment and dementia. Development of dementia in essential tremor is associated with loss of functional ability and a doubling of mortality rate. This prospective, longitudinal study comprehensively reports the prevalence and incidence of, and the annual rates of conversion to, mild cognitive impairment and dementia in an essential tremor cohort., Methods: Patients underwent detailed cognitive assessments and were assigned diagnoses of normal cognition, mild cognitive impairment, or dementia. There were 222 patients at baseline (mean age = 79.3 ± 9.7 years), and 177 patients participated in follow-up evaluations at 18, 36, 54, and 72 months (mean years of observation = 5.1 ± 1.7). Data were compared to those of historical controls and Parkinson disease patients., Results: The cumulative prevalence of dementia and average annual conversion rate of mild cognitive impairment to dementia were 18.5% and 12.2%, nearly three times higher than rates in the general population, and approximately one half the magnitude of those reported for Parkinson disease patients. The cumulative prevalence of mild cognitive impairment (26.6%) was almost double that of the general population, but less than that in Parkinson disease populations., Interpretation: We present the most complete exposition of the longitudinal trajectory of cognitive impairment in an essential tremor cohort yet presented. The prevalence of and conversion rates to dementia in essential tremor fall between those associated with the natural course of aging and the more pronounced rates observed in Parkinson disease. ANN NEUROL 2024;95:1193-1204., (© 2024 The Authors. Annals of Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Essential tremor (ET) is one of the most common tremor disorders and can be disabling in its affect on daily activities. There have been major breakthroughs in the treatment of tremor and ET is the subject of important ongoing research. This review will present recent advancements in the epidemiology, genetics, pathophysiology, diagnosis, comorbidities, and imaging of ET. Current and future treatment options in the management of ET will also be reviewed. The need for continued innovation and scientific inquiry to address the unmet needs of persons of ET will be highlighted., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Dr. Okelberry and Dr. Lyons have nothing to disclose. Dr. Pahwa serves as a consultant for Abbott, AbbVie, ACADIA, Acorda, Allevion, Amneal, Appello, Biogen, BioVie, CalaHealth, Convatec, Fasikl, Genetech, Insightec, Jazz, Keiferx, Kyowa, Lundbeck, Merz, Mitsubishi Tanabe, Neurocrine, Ono, PhotoPharmics, PSG, Reagenxbio, Sage, Sun Biopharma, Supernus, and UCB. He receives research support from Abbott, AbbVie, Alexza, Amylyx, Annovis, ASK Bio, Attune, Biogen, Bluerock, Bukwang, Cerevance, Cerevel, EIP, Fasikl, GemVax, Global Kinetics, Inhibikase, Intra-cellular Therapies (ITI), Jazz, Michael J Fox Foundation, Neurocrine, Neuroderm, ONO, Parkinson's Foundation, Praxis, Roche, Sage, Scion, Sun Pharma, Teva, Theravance, and UCB., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Essential tremor (ET) is a neurological disease characterized by action tremor in upper arms. Although its high heritability and prevalence worldwide, its etiology and association with other diseases are still unknown., Method: We investigated 10 common spinocerebellar ataxias (SCAs), including SCA1, SCA2, SCA3, SCA6, SCA7, SCA8, SCA12, SCA17, SCA36, dentatorubral-pallidoluysian atrophy (DRPLA) in 92 early-onset familial ET pedigrees in China collected from 2016 to 2022., Result: We found one SCA12 proband carried 51 CAG repeats within PPP2R2B gene and one SCA3 proband with intermediate CAG repeats (55) with ATXN3 gene. The other 90 ET probands all had normal repeat expansions., Conclusion: Tremor can be the initial phenotype of certain SCA. For early-onset, familial ET patients, careful physical examinations are needed before genetic SCA screening., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Introduction: Essential tremor (ET) patients may exhibit a variety of non-motor features, including cognitive decline and depressive symptoms. Studies of several neurodegenerative diseases link depression to cognitive decline, suggesting depression is an early marker of dementia. We examined whether baseline depressive symptoms predict incident dementia in elders with ET., Methods: Hundred and forty-one ET cases aged 70 years or older at baseline, enrolled in a prospective study of cognitive performance, took part in evaluations at baseline and at 18, 36, 54, and 72 months. Participants completed the Geriatric Depression Scale (GDS), a 30-item self-report measure of depressive symptoms, and a battery of neuropsychological tests and functional assessments, from which we derived cognitive diagnoses at each evaluation. Cox proportional hazards regression equations determined incident dementia risk based on participants' baseline depression scores., Results: Mean baseline age was 81.5 ± 6.7 years. Higher baseline GDS scores were associated with increased risk of dementia in an unadjusted model (hazards ratio [HR] = 1.11, 95% confidence interval [CI] = 1.02-1.20, p = 0.01) and after controlling for baseline age, education, number of medications, and tremor onset age (HR = 1.13, 95% CI = 1.02-1.25, p = 0.02)., Conclusion: Baseline depression scores predicted incident dementia in elders with ET. With each one-point increase in baseline depression score, there was a 13% increase in incident dementia risk. Given the published data that reported depression may be twice as high in elders with ET compared to controls, this association is particularly worrisome in the ET population., (© 2024 S. Karger AG, Basel.)

Background and Purpose: Essential tremor (ET) is one of the most common neurological disorders, but information on treatment pattern is still scant. The aim of this study was to describe the demographic and clinical characteristics, treatment patterns, and determinants of drug use in patients with newly diagnosed ET in France and the United Kingdom., Methods: Incident cases of ET diagnosed between January 1, 2015 and December 31, 2018 with 2 years of follow-up were identified by using The Health Improvement Network (THIN®) general practice database. During the follow-up, we assessed the daily prevalence of use and potential switches from first-line to second-line treatment or other lines of treatment. Logistic regression models were conducted to assess the effect of demographic and clinical characteristics on the likelihood of receiving ET treatment., Results: A total of 2957 and 3249 patients were selected in the United Kingdom and France, respectively. Among ET patients, drug use increased from 12 months to 1 month prior the date of index diagnosis (ID). After ID, nearly 40% of patients received at least one ET treatment, but during follow-up drug use decreased and at the end of the follow-up approximately 20% of patients were still on treatment. Among treated patients, ≤10% maintained the same treatment throughout the entire follow-up, nearly 20% switched, and 40%-75% interrupted any treatment. Results from the multivariate analysis revealed that, both in France and the United Kingdom, patients receiving multiple concomitant therapies and affected by psychiatric conditions were more likely to receive an ET medication., Conclusion: This study shows that ET is an undertreated disease with a lower-than-expected number of patients receiving and maintaining pharmacological treatment. Misclassification of ET diagnosis should be acknowledged; thus, results require cautious interpretation., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background: Tremors other than those associated with Parkinson's disease (non-parkinsonian tremor) are commonly observed in clinical settings. However, their frequency and clinical characteristics have rarely been reported., Objectives: To classify non-parkinsonian tremors based on the consensus statement on the classification of tremors, from the task force of the International Parkinson and Movement Disorder Society published in 2018., Methods: A prospective registry at a tertiary care teaching institute., Results: A total of 475 patients with non-parkinsonian tremors were recruited for the study. 67.57% (n = 321) of our patients were male and a family history of tremor was present in 20.84% (n = 99) of patients. Dystonic tremor (DT) was the most common non-parkinsonian tremor (33.26%). 27.78% of patients fulfilled the new classification criteria for essential tremor, with 13.47% classified as pure ET (ET) and 14.31% exhibiting neurological soft signs, leading to the classification of ET plus (ETP). Patients with ETP had more family history (57.35%) [vs DT (26.48%, p = 0.00004) and ET (10.93%, p = 0.00003], longer duration of disease [mean ± standard deviation (SD) = 9.53 ± 8.64 years] [vs DT (5.60 ± 5.93, p = 0.0003) and ET (6.38 ± 5.97, p = 0.01) years], and more severe tremor as measured by the essential tremor rating assessment scale total score [mean ± SD = 27.42 ± 11.70] [vs DT (23.50 ± 8.62, p = 0.007) and ET (22.12 ± 8.19, p = 0.007)] compared with patients with DT and ET., Conclusions: DT was the most common cause of non-parkinsonian tremor in our registry followed by essential tremor syndrome. ETP was more common than ET., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2023 The Author(s).)

Background: Although essential tremor (ET) is often divided into familial and sporadic cases, few data compare the evolution of clinical features in these groups over time. Leveraging data from a prospective, longitudinal study, we present analyses of the evolution of a broad range of cognitive, motor (i.e., tremor, tandem gait) and other features (e.g., disability) of ET., Methods: Sixty-six familial and 23 sporadic ET cases completed in-home evaluations at baseline and 18, 36, and 54-month follow-ups. Assessments included detailed neuropsychological testing and videotaped neurological examinations. Analyses compared the longitudinal course of 16 clinical features in familial and sporadic cases., Results: Baseline mean age was 75.2 ± 8.8 years and mean observation period was 4.7 ± 0.3 years. Tremor onset age was lower and childhood onset more common in familial than sporadic cases (p's = 0.02). Longitudinal analyses revealed no significant differences between clinical features displayed by familial and sporadic cases, or differences between the patterns of change in clinical features observed in these groups across time. Sporadic cases' daily activity skills declined significantly, whereas familial cases' did not, p's = 0.04 and 0.34, respectively; however, this finding was non-significant when controlling for false discovery rate. Several additional non-significant trends were noted., Conclusion: Familial and sporadic ET cases differed in onset age, and in the prevalence of childhood tremor onset. Although a number of interesting trends were observed, no significant differences in the evolution of clinical features over time in patients with and without a family history of ET were revealed., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Essential tremor (ET) is a highly prevalent neurological disease. Age of onset can occur anytime between childhood and advanced age. Tremor generally starts insidiously. Age of onset is a particularly important data item in clinical and epidemiological research. In general, these data are self-reported by ET cases. A fundamental question is whether ET cases reliably report their age of onset., Methods: In this prospective, epidemiological study of 125 ET cases, self-reported age of onset data were collected at regular 18 months intervals over four time points., Results: The correlation between self-reported age of onset was high - intra-class correlation coefficient = 0.972 (95% confidence interval = 0.962-0.980, p < 0.001). However, agreement was not perfect. Approximately 20-25% of participant's reports at different time intervals differed by as much as 10 years, and approximately 10% of participant's reports differed by as much as 20 years., Conclusions: There was a robust correlation between self-reports of age of onset. Yet in a not-insignificant number of cases, there were considerable differences, some of which were substantial. These findings have broad implications for development of diagnostic algorithms, data stratification schemes, and analyses that assess correlations between biomarker data and clinical features (e.g., disease duration)., (© 2022 S. Karger AG, Basel.)

Introduction: Essential tremor (ET) is the most common tremor disorder, estimated to affect 7 million individuals in the USA. There is little empirical evidence on comorbidities among this population beyond higher prevalence of brain-related and stress-related disorders. This study aims to examine differences in the prevalence of the 31 Elixhauser comorbidities among ET patients compared to statistically similar control patients., Methods: An extract from Optum's de-identified Clinformatics® Data Mart Database (CDM) from 2018 to 2019 of adults aged 40-80 years with at least one claim with an ET diagnosis was propensity score matched to controls. Logistic regression was used to generate doubly robust adjusted odds ratios for each of the 31 Elixhauser comorbidities., Results: In these analyses, ET patients had significantly greater adjusted odds of depression, alcohol abuse, and other neurological disorders, as well as chronic pulmonary disease, renal failure, hyperthyroidism, and cardiac arrhythmias relative to controls. They also had lower odds of uncomplicated diabetes, congestive heart failure, metastatic cancer, paralysis, peripheral vascular disease, and fluid and electrolyte disorders., Conclusion: A number of recent studies, including our own, suggest that psychiatric, neurologic, and stress-related disorders may be more prevalent among ET patients than controls. Additional differences in the prevalence of a range of medical comorbidities have also been variably reported across studies, suggesting that some combination of these might be more prevalent. Further studies would be of value in sorting through these associations., (© 2023 S. Karger AG, Basel.)

Introduction: Essential tremor (ET) affects approximately 7 million people in the USA, yet public recognition of the disease and its impact remain low., Methods: A retrospective observational study examined US claims data from 2015 to 2019 using the Compile database. ET diagnoses were captured using longitudinal data from 2015 to 2019 and for the year 2019, with diagnosis estimates extrapolated to the general US population. Confirmed ET was identified by an ET diagnosis code with at least two relevant prescriptions or by two diagnosis codes for ET and unspecified tremor at least 90 days apart. Comorbidity and treatment use data were extracted, and medication compliance and 2-year treatment persistence were assessed as measures of treatment adherence., Results: A total of 1,336,183 patients with ET diagnoses codes were identified from 2015 through 2019, corresponding to 2,226,971 projected US diagnoses. In 2019, 128,263 patients had a confirmed ET diagnosis, corresponding to 213,772 projected US confirmed diagnoses. Of these, 96% had at least one comorbidity, and 64% received at least one pharmacologic treatment. Propranolol (24%) and primidone (20%) comprised the most common ET prescriptions. Two-year medication discontinuation rates were approximately 40%., Conclusion: Our findings revealed that 1 million people were diagnosed and sought treatment for ET in the USA from 2015 to 2019. Projected population estimates of approximately 2 million people diagnosed suggest a further 1 million remain untreated. Our findings highlight the complexity of patient care in ET, complicated by delayed diagnoses, multiple comorbidities, and lack of effective and tolerable therapies that can mitigate treatment adherence limitations., (© 2022. The Author(s).)

Background: There are no prospective, longitudinal studies investigating patterns of medication use among essential tremor (ET) patients. Our goal was to fill this knowledge gap. We also had a unique opportunity to examine medication use patterns primarily among elders with longstanding ET. We hypothesized that by the time ET patients reach advanced ages, medication changes would be uncommon - that is, they may have reached some kind of equipoise., Methods: A prospective, longitudinal cohort of ET cases was evaluated across three time points. Cases were not ascertained from a treatment setting, thereby removing important selection biases. Each reported current medications and dosages of each., Results: There were 144 cases (mean baseline age = 76.1 ± 9.4 years). The mean observation period = 2.9 ± 0.2 years. Primidone and propranolol were the most commonly used medications, although almost one-half of cases (44.4%) reported using neither during this period. A third of primidone users (33.3%) and a quarter of propranolol users (24.6%) reported changes in use vs. nonuse during the observation period. The majority of our cases made some change in their daily medication dosage during the course of the study - 73.3% of primidone users and 57.9% of propranolol users., Conclusion: In this prospective, longitudinal study, use vs. nonuse and daily dosage of both primidone and propranolol fluctuated across time for a sizable proportion of ET cases. Even among elders with chronic, longstanding ET, there is considerable ongoing medication adjustment, underscoring the need to improve the medication situation for ET patients., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: The construct of Essential Tremor plus (ET-plus) refers to patients who also have rest tremor and/or mild neurologic signs of unknown significance. It is unclear whether soft signs represent confounding factors or are useful in suspecting an alternative condition., Methods: Using a Bayesian approach to ET-plus patients recruited in The ITAlian tremor Network (TITAN), we analyzed the probability that these patients do not have ET., Results: The data of 274 ET-plus patients were extracted from the TITAN database. The majority of patients (240/274; 87.5%) had a single soft sign. The post-test probability of not having ET was different according to the specific soft sign: namely, 0.64 (rest tremor); 0.46 (questionable dystonia); 0.85 (questionable bradykinesia); 0.19 (soft gait impairment); and 0.09 (questionable cognitive issues). In patients with multiple soft signs, the post-test probability of not having ET was higher than 50% for 7 out of 11 combinations, accounting for 44.1% of subjects. Overall, the post-test probability of not having ET was higher than 50% in up to 71.5% of ET-plus patients., Discussion: We have here shown that: 1) the soft signs differently contribute in modulating the probability that a patient does not have ET; and 2) the effect of multiple soft signs are not always addictive. Future studies are needed to collect prevalence figures of soft signs in different neurological disorders as well as in the elderly and to calculate their value in predicting the development of an alternative tremor syndrome., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Across the world, Essential Tremor (ET) is the most common tremor diagnosis but up to half of these diagnoses are inaccurate. The misdiagnosis rate is particularly high in late-onset ET, when tremor begins after the age of 60 years. Currently, ET is reported to affect 5.5% of those over 65 years old and 21.7% aged over 95 but there is emerging evidence that late-onset ET has associations with dementia, mortality and more rapid progression. With ageing populations, and a range of new surgical treatments for ET, there is urgent need to clarify whether the clinical manifestations of late-onset ET are the same as for earlier-onset ET. This scoping review used MEDLINE, EMBASE and CINAHL as the information sources of published peer-reviewed research articles between 2011 and 2021. Analysis was done by narrative synthesis. 14 relevant papers were retrieved from studies conducted in Denmark, India, Italy, Germany, Spain and the US and, together, they comprised 7684 participants in total. Compared to older adults with earlier-onset ET, there is evidence that late-onset ET is associated with higher risk of cognitive impairment and dementia, higher mortality rate, faster rate of progression, lack of family history, altered cortical electrical activity, prolonged pupillary responses, and less propensity to demonstrate characteristic alcohol sensitivity. There is evidence that late-onset ET has different clinical manifestations to earlier-onset ET; in particular there is higher risk of dementia and mortality. The prognosis is important for clinicians to consider when selecting candidates for deep brain stimulation surgery and also for advanced care planning., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Abstract: Essential tremor (ET) is one of the most common neurological diseases. Despite this high prevalence, treatment options remain limited. Recent advances in mechanistic research have led to renewed interest in developing newer pharmacotherapeutic agents. Although this is promising, there remains surprisingly little knowledge of the magnitude of the vast ET patient population who would benefit from and/or engage with such agents. This is because the vast bulk of ET patients in the population has milder tremor and does not seek medical care for their tremor. How many of these would be interested in taking a new agent? Hence, the level of unmet need, from the patient perspective and from a public health perspective, is totally unclear. In this article, the author systematically reviews peer-reviewed data on several pertinent questions that relate to this unmet need. The questions are as follows: (1) What proportion of the population of ET cases sees a health care provider for their tremor? (2) What proportion of the ET population has a tremor that is severe enough for them to want to take a medication for their tremor? (3) How do other factors such as age, sex, education, health literacy, and locale affect the receptivity to treatment in ET? (4) What is the patient use of the current ET medications? (5) Is the unmet need greater in some groups of ET cases than others? For each of these questions, available data are carefully reviewed and analyzed, and gaps in knowledge identified for further investigation., Competing Interests: Conflicts of Interest and Source of Funding: The author has no conflicts of interest to declare. Study funding: NIH R01 NS117745, R01 NS086736, and R01 NS088257., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

The diverse clinical manifestation of essential tremor (ET) has led to the question whether the different phenotypes may affect the clinical outcome and progression. This study aimed to estimate the clinical characteristics and natural history of ET and ET-plus. A total of 221 patients with ET were included, 117 (52.9%) reclassified as ET and 104 (47.1%) as ET-plus. Patients with ET-plus were significantly older in age at onset (P < 0.001); had a higher frequency of cranial tremors (P < 0.001), neurological comorbidities (P < 0.001) and psychiatric comorbidities (P = 0.025); more tremor progression (P < 0.001); and poorer response to medical treatment (P < 0.001) compared to ET patients. Regression analysis revealed that late-onset tremor (OR 11.02, 95% CI 2.79-43.53), neurological comorbidities (OR 3.38, 95% CI 1.56-7.31), psychiatric comorbidities (OR 4.29, 95% CI 1.48-12.44), cranial tremors (OR 2.10, 95% CI 1.02-4.30), and poor response to medical treatment (OR 3.67, 95% CI 1.87-7.19) were associated with ET-plus diagnosis. ET and ET-plus differ in the age of onset, tremor distribution, comorbidities, treatment response rate, and progression. Identifying the ET phenotypes may increase the clinical value in therapeutic strategies and clinical research in the future., (© 2022. The Author(s).)

Background: Essential tremor (ET) is one of the most common movement disorders worldwide, yet the size of the pediatric ET population is not well understood. The objective of this review was to identify, evaluate, and synthesize evidence describing the epidemiology of pediatric ET in the United States published between 2010 and 2020., Methods: The authors searched MEDLINE, Embase, and the Cochrane Database of Systematic Reviews using terms related to ET, epidemiology, and pediatric patients. Eligibility criteria included observational studies that reported primary data on pediatric prevalence or incidence of ET or age of onset/diagnosis of ET. A total of 562 unique articles were identified for screening., Results: The review did not identify any studies that reported information on pediatric prevalence or incidence of ET, or age of ET diagnosis among nonpediatric patients. A total of 10 samples were identified, all of which described age of ET onset that ranged from 27.0 years to 56.7 years among 9 adult populations (weighted mean of 41.6 years) and 9.7 years in a single pediatric sample. One adult sample reported that 13% of all ET cases reported onset by age 14, and 21.8% of all ET cases reported onset by age 18., Discussion: There is a notable lack of recent data describing the incidence and prevalence of pediatric ET in the United States. Many children who present with symptoms of ET may not be diagnosed until later in life, and an increased awareness of pediatric ET could allow for early identification and monitoring of these patients., Competing Interests: MG and AEL are employees of Sage Therapeutics, Inc., and own stock and stock options. KL reports having received consulting fees from Abbott and Sage and is the President of the International Essential Tremor Foundation., (Copyright: © 2022 The Author(s).)

Essential tremor (ET) is the most common neurological cause of tremor affecting adult humans affecting about 6% of those over age 65 years. In the United States, dementia has a prevalence of 15% in those age 68 and older. Overlap of the two conditions is therefore not surprising. Several studies report mild subclinical cognitive dysfunction in non-demented people with ET, likely related to overactivity of fronto-cerebellar circuitry involved in tremor pathophysiology. Frontal/executive dysfunction is often though not exclusively noted, and some studies have even shown areas of cognitive strengths. Mild cognitive impairment (MCI) is impairment which a person is aware of but does not interfere significantly with daily activities. While MCI has been considered to presage dementia this is not necessarily the case, as some persons with MCI revert to normal cognition in follow-up. Dementia is a clinical syndrome with cognitive impairment interfering with daily activities. Population-based and clinic-based studies have shown mixed results regarding rates of MCI in ET. A handful of studies have looked at development of dementia in ET with differing results. Brain pathology studies in ET and dementia or investigating Alzheimer-type pathology have thus far been unrevealing. There is evidence by some investigators supporting a greater risk of dementia for those having older onset tremor, while those having ET at a younger age faring at least as well as controls regarding risk of cognitive impairment and dementia. At present the evidence is inconclusive that ET as a group are at a greater risk of developing dementia., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Essential tremor (ET) is a highly prevalent neurological disease that frequently runs in families. A recent and controversial proposal is to separate ET patients into two distinct groups - ET versus ET-plus. If this were a valid construct, one would expect in familial aggregation studies to observe that ET-plus would cluster in some families yet be absent in others, rather than being randomly distributed across families. We examined whether there is evidence of familial aggregation of ET-plus., Methods: Probands (n = 84 [56 ET-plus and 28 ET]) and their first- and second-degree relatives (n = 182 and 48) enrolled in a genetics study. χ2 and generalized estimating equations (GEE) tested associations between probands' ET-plus status and the ET-plus status of their relatives., Results: χ2 analyses revealed that ET-plus was no more prevalent in relatives of probands diagnosed with ET-plus than in relatives of probands diagnosed with ET, p > 0.05. Restricting relatives to first-degree relatives similarly did not detect a significant association (p = 0.88). GEE yielded similar results (respective p's = 0.39 and 0.81)., Conclusion: The data demonstrate that ET-plus does not seem to aggregate in families. As such, they do not lend support to the notion that ET-plus is a valid biological construct., (© 2022 S. Karger AG, Basel.)

Sex and gender-based differences in epidemiology, clinical features and therapeutical responses are emerging in several movement disorders, even though they are still not widely recognized. In this chapter, we summarize the most relevant evidence concerning these differences in Parkinson's disease, essential tremor, dystonia and chorea. Indeed, both sex-related biological (hormonal levels fluctuations) and gender-related variables (socio-cultural and environmental factors) may differently impact symptoms manifestation and severity, phenotype and disease progression of movement disorders on men and women. Moreover, sex differences in treatment responses should be taken into account in any therapeutical planning. Physicians need to be aware of these major differences between men and women that will eventually have a major impact on better tailoring prevention, treatment, or even delaying progression of the most common movement disorders., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Essential Tremor (ET), by definition, is a disorder of movement. Yet over the years, epidemiologic, clinical, pathologic, and neuroimaging studies have converged to reveal a cognitive side of ET. The cognitive symptoms in ET are heterogeneous and are likely to reflect heterogeneous underlying mechanisms. In this chapter, we review and synthesize a diverse set of studies from both population-based settings to cohorts with more detailed investigations into cognition to consider the various mechanisms by which cognitive symptoms may emerge in a subset of individuals with ET. As part of our analysis, we consider questions surrounding ET diagnosis and the possibility of comorbid disease as potential factors that, upon closer examination, appear to strengthen the argument in favor of ET as a risk factor for dementia. Importantly, we also consider the clinical relevance of cognitive impairment in ET. While ET is not universally characterized by significant cognitive deficits, the data from epidemiological, cognitive, neuroimaging, and postmortem neuropathologic studies converge to reveal an increased risk for cognitive impairment and dementia among individuals with ET. We conclude by offering directions for future research, and a neurocognitive framework with which to consider existing findings and to use in the design of novel studies dedicated to clarifying the basis, nature, and course of cognitive impairments in ET., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Essential tremor (ET) is one of the most common movement disorders, with a reported >60 million affected individuals worldwide. The definition and underlying pathophysiology of ET are contentious. Patients present primarily with motor features such as postural and action tremors, but may also have other non-motor features, including cognitive impairment and neuropsychiatric symptoms. Genetics account for most of the ET risk but environmental factors may also be involved. However, the variable penetrance and challenges in validating data make gene-environment analysis difficult. Structural changes in cerebellar Purkinje cells and neighbouring neuronal populations have been observed in post-mortem studies, and other studies have found GABAergic dysfunction and dysregulation of the cerebellar-thalamic-cortical circuitry. Commonly prescribed medications include propranolol and primidone. Deep brain stimulation and ultrasound thalamotomy are surgical options in patients with medically intractable ET. Further research in post-mortem studies, and animal and cell-based models may help identify new pathophysiological clues and therapeutic targets and, together with advances in omics and machine learning, may facilitate the development of precision medicine for patients with ET., (© 2021. Springer Nature Limited.)

Background: Essential tremor (ET) is among the most prevalent movement disorders. Comprehensive reviews of disease prevalence were published in 1998 and 2010 but not since then. We reviewed the prevalence of ET in population-based epidemiological studies, derived a precise summary estimate of prevalence from these studies, and examined differences in prevalence across studies. We used two methods: a descriptive-analytical approach and a meta-analysis., Methods: A PUBMED search yielded 14 published papers since the 2010 review., Results: There were 42 population-based prevalence studies (23 countries and 6 continents). In a meta-analysis, pooled prevalence (all ages) = 1.33%, with statistically significant heterogeneity across studies (I 2 = 99.3%, p < 0.0001). In additional descriptive analyses, median crude prevalence (all ages) = 0.4% and mean = 0.67%. Prevalence increased markedly with age, and especially with advanced age. In the meta-analysis, prevalence (age ≥ 65 years) = 5.79%, and in descriptive analyses, median crude prevalence (age ≥ 60-65) = 5.9% and mean = 8.0%. In the oldest age groups, median prevalence = 9.3%, with several studies reporting values >20%. The prevalence increased by 74% for every decade increase in age (p < 0.0001). Gender did not impact the prevalence of ET (p = 0.90)., Discussion: Precise prevalence estimates are important because they form the numerical basis for public health initiatives and offer clues about underlying biological factors of mechanistic importance. The prevalence of ET among those age ≥ 65 is similar to that reported for Alzheimer's disease in elders, suggesting that ET may be the most common neurodegenerative disease., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2021 The Author(s).)

Background: Patients with essential tremor (ET) have an increased risk of cognitive impairment, yet little is known about the predictors of cognitive decline in these patients. Exposures to infectious agents throughout the lifespan may impact the later development of cognitive impairment. For example, high Infection exposure has been associated with lower cognitive performance in Alzheimer's and Parkinson's disease. However, this predictor has not been examined in ET., Objectives: To determine whether a higher baseline infection burden is associated with worse cognitive performance at baseline and greater cognitive decline over time in an ET cohort., Method/design: 160 elderly non-demented ET participants (80.0 ± 9.5 years) underwent an extensive cognitive evaluation at three time points. At baseline, participants completed an infection burden questionnaire (t-IBQ) that elicited information on previous exposure to infectious agents and number of episodes per disease. Analysis of covariance and generalized estimated equations (GEEs) were used., Results: Overall, infection burden was not associated baseline cognitive performance. Adjusted GEE models for repeated measures yielded a significant time interaction between moderate infection burden at baseline and better performance in the attention domain over time ( p = 0.013). Previous history of rubella was associated with faster rate of decline in visuospatial performance ( p = 0.046)., Conclusion: The data were mixed. Moderate self-reported infection burden was associated with better attention performance over time. Self-reported history of rubella infection was related to lower visuospatial performance over time in this cohort. Follow-up studies with additional design elements would be of value., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2021 The Author(s).)

Background: Essential tremor (ET) is one of the most common neurological disorders that impairs quality of life and leads to disability and social handicap. It was estimated that approximate 0.9% of people worldwide were affected by ET. The last decade has seen new investigations on the epidemiology of ET, enabling us to provide an up-to-date estimation of ET prevalence, with emphasis on age and sex., Methods: We searched PubMed, Medline, Embase and Global Health for studies that reported the prevalence of ET in the general population. Potential sources of heterogeneity were examined by age-adjusted meta-regression. The age- and sex-specific prevalence of ET was constructed with a multilevel mixed-effects meta-regression., Results: A total of 29 articles were included in our systematic review and meta-analysis. The prevalence of ET increased dramatically with advancing age, where the prevalence estimate in people aged under 20 years was 0.04% (95% confidence interval [CI] = 0.00-0.29) and that in elderly aged 80 years and above was 2.87% (95% CI = 1.07-7.49). ET was consistently more common in males than in females. In 2020, the overall prevalence of ET in the general population was 0.32% (95% CI = 0.12-0.91), and the prevalence was higher in males (0.36%, 95% CI = 0.14-1.03) than in females (0.28%, 95% CI = 0.11-0.79). In 2020, the number of people affected by ET was 24.91million (95% CI = 9.51-70.92), among whom 56% were males., Conclusions: This study provides an up-to-date estimation of ET prevalence in the general population throughout the whole life span, with emphasis on age and sex. The adoption of an internationally acknowledged diagnostic strategy is prompted in future epidemiological investigations., Protocol Registration: PROSPERO (CRD42020203979)., Competing Interests: Competing interests: IR is co-Editor-in-Chief of the Journal of Global Health. To ensure that any possible conflict of interest relevant to the journal has been addressed, this article was reviewed according to best practice guidelines of international editorial organisations. The author completed the ICMJE Unified Competing Interest form (available on request from the corresponding author). The authors declare no competing interests., (Copyright © 2021 by the Journal of Global Health. All rights reserved.)

Purpose of the Review: Patients diagnosed with essential tremor (ET) report frequent sleep complaints. This review focuses on the main findings of studies addressing sleep features in patients diagnosed with ET, updating previously reported information. Bad quality of sleep and excessive daytime somnolence are very frequent in patients with ET, although the effects of the drugs used for the therapy of ET could contribute to these complaints. REM sleep behavior disorder, restless legs, insomnia, and nocturia are frequent complaints as well. There is a lack of studies addressing polysomnographic features of ET.

Background: Several feasibility studies and a randomized, controlled, multicenter trial have demonstrated the safety and efficacy of unilateral transcranial magnetic resonance-guided focused ultrasound (FUS) lesioning of the ventral intermediate thalamic nucleus in treating essential tremor., Objective: To evaluate the safety and efficacy of FUS thalamotomy in a Japanese patient cohort through a prospective, multicenter, single-arm confirmatory trial., Methods: A total of 35 patients with disabling refractory essential tremor underwent unilateral FUS thalamotomy and were followed up for 12 post-treatment months. Safety was measured as the incidence and severity of treatment-related adverse events. Efficacy was measured as the tremor severity and quality of life improvements using the Clinical Rating Scale for Tremor and Questionnaire for Essential Tremor., Results: The mean skull density ratio (SDR) was 0.47. There was a significant decrease in the mean postural tremor score of the treated hand from baseline to 12 mo by 56.4% (95% CI: 46.7%-66.1%; P < .001), which was maintained at last follow-up. Quality of life improved by 46.3% (mean overall Questionnaire for Essential Tremor score of 17.4 [95% CI: 12.1-22.7]) and there were no severe adverse events. The most frequent adverse event was gait disturbance and all events resolved., Conclusion: Unilateral FUS thalamotomy allowed significant and sustained tremor relief and improved the quality of life with an outstanding safety profile. The observed safety and efficacy of FUS thalamotomy were comparable to those reported in a previous multicenter study with a low SDR, and inclusion of the low SDR group did not affect effectiveness., (© Congress of Neurological Surgeons 2021.)

Purpose: Essential tremor (ET) is the most common movement disorder in which dysautonomia symptoms can be present. We aimed to evaluate the presence of orthostatic hypotension (OH) and its relationship with the clinical features., Patients and Methods: Forty-four elderly patients with ET and 118 healthy elderly controls were included. OH was assessed via the head-up tilt table test and defined, according to the change in position, as a drop of at least 20 mmHg in systolic blood pressure and/or 10 mmHg in diastolic blood pressure. Tremor severity was evaluated using the Fahn-Tolosa-Marin Tremor Rating (FTMTR) Scale. All patients underwent geriatric comprehensive assessment., Results: There were no differences between the controls and patients with ET regarding age and gender. The mean age was 72.8±6.1, the mean disease duration 19.1±13.5 years and the mean FTMTR score was 30.9±17.1 in patients with ET. The frequency of OH at the 1st minute in patients with ET was higher than in controls (31.8% vs 17.8%, p=0.046). Furthermore, the frequency of jaw tremor in patients with OH was higher than in those without OH (35.7% vs 6.7%, p=0.025). About 28.6% of ET patients with OH had orthostatic symptoms., Conclusion: We demonstrated that ET patients, particularly those with jaw tremors, had OH and that most of them were asymptomatic. Therefore, in order to protect patients from complications related to OH, it would be appropriate to evaluate OH in the follow-up and treatment of elderly patients with ET., Competing Interests: The authors report no conflict of interest in this work., (© 2021 Kaya et al.)

Background: While considered a safe operation, deep brain stimulation (DBS) has been associated with various morbidities. We assessed differences in postsurgical complication rates in patients undergoing the most common types of neurostimulation surgery., Methods: The National Readmission Database (NRD) was queried to identify patients undergoing neurostimulation placement with the diagnosis of Parkinson disease (PD), epilepsy, dystonia, or essential tremor (ET). Demographics and complications, including infection, pneumonia, and neurostimulator revision, were queried for each cohort and compiled. Readmissions were assessed in 30-, 90-, and 180-day intervals. We implemented nearest-neighbor propensity score matching to control for demographic and sample size differences between groups., Results: We identified 3230 patients with Parkinson disease, 1289 with essential tremor, 965 with epilepsy, and 221 with dystonia. Following propensity score matching, 221 patients remained in each cohort. Readmission rates 30-days after hospital discharge for PD patients (15.5 %) were significantly greater than those for ET (7.8 %) and seizure patients (4.4 %). Pneumonia was reported for PD (1.6 %), seizure (3.3 %) and dystonia (1.7 %) patients but not individuals ET. No PD patients were readmitted at 30-days due to dysphagia while individuals treated for ET (6.5 %), seizure (1.6 %) and dystonia (5.2 %) were. DBS-revision surgery was performed for 11.48 % of PD, 6.52 % of ET, 1.64 % of seizure and 6.90 % of dystonia patients within 30-days of hospital discharge., Conclusion: 30-day readmission rates vary significantly between indications, with patients receiving DBS for PD having the highest rates. Further longitudinal studies are required to describe drivers of variation in postoperative outcomes following DBS surgery for different indications., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: The objective of this study was to determine the prevalence of the GGC-repeat expansion in NOTCH2NLC in whites presenting with movement disorders., Methods: We searched for the GGC-repeat expansion in NOTCH2NLC using repeat-primed polymerase chain reaction in 203 patients with essential tremor, 825 patients with PD, 194 patients with spinocerebellar ataxia, 207 patients with "possible" or "probable" MSA, and 336 patients with pathologically confirmed MSA. We also screened 30,008 patients enrolled in the 100,000 Genomes Project for the same mutation using ExpansionHunter, followed by repeat-primed polymerase chain reaction. All possible expansions were confirmed by Southern blotting and/or long-read sequencing., Results: We identified 1 patient who carried the NOTCH2NLC mutation in the essential tremor cohort, and 1 patient presenting with recurrent encephalopathy and postural tremor/parkinsonism in the 100,000 Genomes Project., Conclusions: GGC-repeat expansion in NOTCH2NLC is rare in whites presenting with movement disorders. In addition, existing whole-genome sequencing data are useful in case ascertainment. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society., (© 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Background: A recent study on early onset Parkinson's disease (PD) revealed that NUS1 is a risk gene for PD. Clinically, essential tremor (ET) is closely related to PD. In this study, we aimed to detect NUS1 variants and assess the effect of those variants on patients with ET., Methods: The 5 coding regions and the exon-intron boundaries of NUS1 were directly sequenced in 395 patients with ET and an equal number of healthy controls, matched for age and sex. The function of variants was assessed by pathogenic predictive software programs. Genetic analysis of variants was used to evaluate susceptibility to ET., Results: A total of 6 exonic variants were identified, including 3 synonymous and 3 missense variants. The non-synonymous variants were predicted to be tolerable. No variants had significant association with ET (none of the p-values were less than 0.05, using Fisher's exact test)., Conclusion: Our study suggested that NUS1 variants may not contribute to the risk of ET., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: Essential tremor (ET) patients presenting tremor in the midline structures may be a distinct subtype of the syndrome. Therefore, we sought to explore the clinical manifestations, especially non-motor symptoms (NMS) of Chinese ET patients with midline tremor (MT)., Methods: In the cross-sectional study, we grouped 290 definite or probable ET patients based on their MT conditions. The NMS in ET patients were evaluated using the NMS scale (NMSS). NMS and other clinical correlates were then compared among subgroups with, and without MT., Results: We revealed that 39.0%, 27.6%, and 6.9% of the patients respectively had neck, voice, and facial tremors. With the accumulation of tremor in midline structures, NMS became more severe and prevalent. Logistic regression analyses revealed that factors such as: female gender (OR = 2.164, 95% CI: 1.307-3.583), having least or highest action arm tremor (OR = 2.512, 95% CI: 1.520-4.151), having higher score of sleep/fatigue domain (OR = 1.692, 95% CI: 1.004-2.850) and mood/apathy (OR = 1.926, 95% CI: 1.143-3.246) domain, to be independently associated with MT manifestation., Conclusions: Our study demonstrates the heterogeneity of symptoms in ET patients with MT, especially in prominent NMS. In addition, the discrepancy of NMS between patients with, and without MT provides novel insight into the underlying pathophysiology and therapeutic of ET., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Elevated tissue levels of the tremor-producing neurotoxin, harmane, have been detected in patients with essential tremor (ET) in the USA and Spain. Recently, a study in the Faroe Islands similarly noted an elevation in blood harmane concentrations in probable and definite ET cases. The underlying mechanism is not understood. Possible mechanisms include increased dietary consumption (esp. through cooked meats), impaired metabolism, or increased endogenous production of harmane. To investigate this issue further, we conducted a population-based study in the Faroe Islands to examine meat consumption and meat cooking practices in ET cases and controls., Methods: 1,328 Faroese adults were screened for tremor and 27 ET cases were identified. Meat consumption and meat cooking practices were compared to 200 controls. Detailed data were collected via questionnaires regarding current meat consumption for 14 meat types and meat cooking doneness for 8 meat types. Data were also available on blood harmane concentrations., Results: Current meat consumption was similar in ET cases and controls in 12 out of 14 meat types, with no differences observed after a Bonferroni correction in any meat type; no difference was observed when stratified by gender. No difference was observed in meat doneness between ET cases and controls. Blood harmane concentrations were not correlated with dietary data., Discussion: This is the first population-based study of harmane-linked dietary factors in ET. The study suggests the observed difference in blood harmane in ET is not driven by dietary differences and is likely due to other mechanisms (e.g., impaired metabolism)., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2020 The Author(s).)

Introduction: Essential tremor (ET) is one of the most prevalent movement disorders. The genetic etiology of ET has not been well defined although a significant proportion (≥50%) are familial cases. Linkage analysis and genome-wide association studies (GWASs) have identified several risk variants. In recent years, whole-exome sequencing of ET has revealed several specific causal variants in FUS (p.Q290X), HTRA2 (p.G399S), and TENM4 (c.4324 G>A, c.4100C>A, and c.3412G>A) genes., Objective: To investigate the genetic contribution of these three genes to ET, the protein-coding sequences of FUS, HTRA2, and TENM4 were analyzed in a total of 238 ET patients and 272 controls from eastern China using direct Sanger sequencing., Results: We identified two synonymous coding single nucleotide polymorphisms (SNPs), rs741810 and rs1052352 in FUS, and three previously reported synonymous SNPs, rs11237621, rs689369, and rs2277277 in TENM4. No nonsynonymous exonic variants were identified in these subjects. We found that the frequency of the rs1052352C allele was significantly higher (P = .001) in the ET group than in the control group., Conclusion: Overall, our findings suggest that rs1052352 of FUS might contribute to ET risk in Chinese population., (© 2020 The Authors. CNS Neuroscience & Therapeutics Published by John Wiley & Sons Ltd.)

Background: Essential tremor (ET) is the most common adult movement disorder, characterized by several motor and increasingly well recognized non-motor symptoms. Sensory deficits, such as hearing impairment and olfactory dysfunction, are amongst them. This review analyzes the available evidence of these sensory deficits and their possible mechanistic basis in patients with ET., Method: A PubMed literature search on the topic was performed in the May 2019 database., Results: Nineteen articles on hearing impairment and olfactory dysfunction in ET patients were identified. The prevalence of hearing impairment is higher in ET patients than healthy controls or Parkinson disease. Cochlear pathologies are suggested as the underlying cause, but there is still a lack of information about retrocochlear pathologies and central auditory processing. Reports on olfactory dysfunction have conflicting results. The presence of mild olfactory dysfunction in ET was suggested. Conflicting results may be due to the lack of consideration of the disease's heterogeneity, but according to recent data, most studies do not find prominent evidence of olfactory loss in ET., Conclusion: Although there is increasing interest in studies on non-motor symptoms in ET, there are few studies on sensory deficits, which are of particularly high prevalence. More studies are needed on to investigate the basis of non-motor symptoms, including sensory deficits., Competing Interests: The author has no competing interests to declare., (Copyright: © 2020 The Author(s).)

Background: The International Parkinson and Movement Disorder Society introduced the category of essential tremor (ET)-plus in its new ET classification scheme, but how the clinical correlates of ET-plus differ from those of "pure" ET is unclear. By comparing the clinical characteristics of ET and ET-plus patients, we expect to better understand the impact and invoked questions of the new classification on clinical practice., Methods: We reviewed the medical records of 280 ET syndrome patients in an ongoing cross-sectional study in a Chinese population and reclassified them according to the new criteria. Clinico-demographic characteristics were compared between ET and ET-plus patients. Risk factors of diagnosis of ET-plus were explored using logistic regression., Results: A total of 121 patients (50.8%) were reclassified as having ET and 117 as having ET-plus. ET-plus group was significantly older at tremor onset, less educated, and more likely to have cranial tremor, depression symptom, anxiety symptom, and probable REM sleep behavior disorder. ET-plus group also showed more severe upper limb tremor and cognition impairment. Regression analysis identified four independent risk factors associated with ET-plus: late tremor onset (OR 3.04, 95%CI 1.60-5.79), less educated (OR 0.91, 95%CI 0.85-0.97), severe upper limb tremor (OR 2.46, 95%CI 1.30-4.62), and presence of cranial tremor (OR 2.30, 95%CI 1.20-4.41)., Conclusions: The new classification scheme emphasized that ET syndrome is heterogeneous. ET-plus cannot be seen as a subtype or a diagnosis of ET syndrome, but rather as a placeholder, representing an area of current scientific uncertainty., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: The aim of this study was to investigate potential genetic overlap between essential tremor and Parkinson's disease in a cohort of 825 subjects from an Eastern Chinese population., Methods: A total of 441 Parkinson's disease patients and 384 healthy controls were recruited. The MassARRAY System was used to detect three essential tremor-related single nucleotide polymorphisms. Odds ratio (OR) and 95% confidential interval (CI) were calculated to assess the relationship between polymorphisms and Parkinson's disease susceptibility., Results: Our results demonstrated that the odds ratios of rs3794087 of SLC1A2, rs9652490 of LINGO1, and rs17590046 of PPARGC1A were 0.71 (95% CI = 0.55-0.91), 0.99 (95% CI = 0.78-1.26), and 0.88 (95% CI = 0.62-1.25), respectively., Conclusion: An essential tremor SNP (rs3794087 of SLC1A2) is associated with a decreased risk of PD in the Eastern Han Chinese population, while rs9652490 (LINGO1) and rs17590046 (PPARGC1A) do not show an association., (© 2019 The Authors. CNS Neuroscience & Therapeutics Published by John Wiley & Sons Ltd.)

Introduction: Essential tremor is one of the pathologies with more proportional weight in neurology consultations. However, compared to other neurodegenerative diseases, little is known about its prevalence and, in particular, its incidence and mortality, as well as which are the genetic, environmental, and biological factors of etiological importance., Aims: To present the most relevant data on the descriptive epidemiology (prevalence, incidence, and mortality) of essential tremor. Likewise, some of the main risk or protective factors of this disease (analytical epidemiology) that have been suggested mainly thanks to the data obtained from large population cohorts will be discussed., Development: We analysed the most significant population studies, especially those carried out through door-to-door methodology, and the most relevant analytical studies., Conclusions: Data on the epidemiology of essential tremor are still limited. Estimates of the prevalence of essential tremor vary widely across countries, which makes it difficult to accurately establish its prevalence. Even so, its prevalence increases with age; that is, it is a disease linked to aging. In subjects older than 60 years, essential tremor prevalence would be between 2.3-14.3% (median: 6.3%). However, further studies are needed in this regard, mainly studies of incidence and mortality. In recent years, advances have been made in the knowledge of some environmental factors, such as risk factors, especially the harmanes.

Essential tremor (ET) is a common neurological disorder and the most common movement disorder. Low-level occupational exposure to mercury vapor is known to be a crucial factor that increases the risk of tremor. Dental amalgam is one of the main sources of mercury in those who possess amalgam restorations. However, the relationship between ET and amalgam filling (AMF) is not quite clear. The purpose of this study was to investigate the association between AMF and the risk of ET using a population-based administrative databank. The data for this study were sourced from the Taiwanese National Health Insurance Research Database (NHIRD). A retrospective case-control study was conducted using this databank from 2000 to 2013. Case and control groups were matched by sex, age, urbanization level, monthly income, and Charlson comorbidity index using the propensity score method with a 1:1 ratio. In this study, 3008 cases and 3008 controls were included. The results from this nationwide population-based case-control study did not indicate any association between ET and AMF in Taiwan. Although the results were not significantly statistical, the findings may be worthy to be valued.

Background: The consensus statement by the Task Force on Tremor of the International Parkinson and Movement Disorder Society excludes individuals with "isolated voice tremor" as a clinical variant of essential tremor (ET). This clinical viewpoint presents a rationale for reconsideration of "isolated voice tremor" as a clinical variant of ET., Methods: Evidence from the literature was extracted to characterize the clinical phenotype of "isolated voice tremor," or essential vocal tremor (EVT). Clinical features were extracted from relevant literature available at pubmed.gov using the terms "EVT," "essential voice tremor," "primary voice tremor," and "organic voice tremor.", Results: The average age of onset in those with EVT was older than 60 years (range 19-84 years), with 75-93% being female. The typical duration of vocal tremor ranged from 1 to 13 years (average 6 years). The distribution of structures exhibiting tremor included the larynx, soft palate, pharynx, and base of tongue in the majority of patients, with some exhibiting tremor of the head and respiratory musculature. The condition of tremor occurred during speech and quiet respiration in 74% of individuals. Rate of tremor ranged from 4 to 10 Hz. Nearly 70% reported onset of vocal tremor prior to upper limb involvement. Family history of tremor was reported in 38-42% of individuals., Discussion: Those previously classified with EVT demonstrate a similar familial history, rate, tremor classification, and body distribution of ET. EVT is proposed as a clinical variant of ET in the pattern of onset and progression of body distribution from the midline cranial to spinal neural pathways., Competing Interests: Funding: This work is funded by the NIDCD to conduct research on those with essential tremor and vocal tremor (R01DC016838, Physiologic correlates of vocal tremor affecting those with essential tremor, PI: Barkmeier-Kraemer). Conflict of Interest: The author conducts funded and unfunded research on individuals with vocal tremor relevant to the topic of this viewpoint. Ethics Statement: Not applicable for this category of article., (© 2020 Barkmeier-Kraemer.)

Objectives: To investigate the prevalence and the clinical feature of essential tremor (ET) in a community cohort in Beijing., Methods: Using a door-to-door, two-phase approach, we investigated 2,835 residents aged ≥55 years old from rural, urban, and mountain areas., Results: The prevalence rate of ET was 4.29%, 2.85%, and 2.29% in rural, urban, and mountain areas, respectively. The overall age- and sex-adjusted prevalence was 3.29%. Among those aged ≥75 years, the prevalence rate in the urban area was higher than those in the rural and mountain areas. The prevalence rate increased with age, and the prevalence was higher among men (6.0%) than among women (3.6%). There was a correlation of ET prevalence with age, sex, and habitation area, but not with alcohol, tea drinking, and occupation. Women (25%) with ET were more likely to have head tremor than men (16.9%)., Conclusions: The ET prevalence in the elderly of Beijing was 3.29% which is higher in the urban area and in men., Competing Interests: Nothing to report.

In addition to having postural and kinetic tremor of the upper limbs, some patients with essential tremor (ET) may have head tremor as well as cognitive and psychiatric disorders. We aimed to investigate whether the variable clinical presentation in ET patients, including motor and non-motor symptoms, differs in patients with and without head tremor. We consecutively enrolled 70 patients with a diagnosis of ET. Tremor severity was assessed by means of clinical rating scales. Patients also underwent kinematic recordings of postural and kinetic tremor of the upper limbs based on an optoelectronic system. Several neuropsychological tests were also administered. Finally, we adopted the structured interviews for DSM-IV, SCID-I, and SCID-II to investigate psychiatric and personality disorders. ET patients with upper limb tremor plus head tremor exhibited more severe kinetic tremor of the upper limbs and a higher occurrence of axis I psychiatric disorders than ET patients with upper limb tremor only. Cognitive and other motor and psychiatric features did not differ significantly with respect to tremor distribution. The study findings support the hypothesis that body tremor distribution, i.e., the presence of head tremor, influences the variable clinical presentation of ET. The study results support the notion that cases with head tremor may represent a distinct ET subtype, characterized by a prominent cerebellar involvement, and that psychiatric disorders should be considered as a specific manifestation of ET.

Objective : We aimed to determine the prevalence and risk factors of Essential Tremor (ET) in Edirne and its districts, located in Western Thrace, which is the most western part of Turkey. Methods : In this study, 3008 individuals who could communicate and agreed to participate in the study were evaluated. To obtain the data from the applicants in 30 Family Health Centres in Edirne and its districts, a face-to-face questionnaire that consisted of 37 questions was prepared by the researchers. The questionnaire included general information, questions to evaluate potential concomitant comorbid conditions and questions regarding the symptomatology used in ET diagnosis, as well as questions to evaluate ET severity, was examined with the spiral test. Patients were classified by using the Washington Heights-Inwood Genetic Study of Essential Tremor (WHIGET) diagnostic and clinical evaluation scale. According to the diagnostic criteria for ET (used in participants who were examined and in those whose medical records were reviewed) were similar to those used in astudy conducted in Turkey. Results : Of 3008 individuals, 173 were diagnosed with ET according to the questionnaire results from Edirne and its districts, and the prevalence of ET was 5.8%. Approximately, 43.4% of the patients with ET were male, and 56.6% were female, which was not significantly different (p > 0.05). Participants with tremor related to alcohol withdrawal, hyperthyroidism, anxiety, depression other known causes of tremor were not considered to have ET. Thyroid disease was identified in 0.0% of the cases, and the control group was detected in 1.4%, which was not significantly different (p = 0.170). Psychiatric disease was identified in 0.0% of the cases, and the control group was detected in 1.3%, which was not significantly different (p = 0.271). Conclusions : ET prevalence studies will increase the awareness of the community and provide early diagnosis and treatment, as well as serve as a basis to reduce morbidity and improve the quality of life.

Essential tremor is one of the most common movement disorders in adults and can affect both children and adults. An updated consensus statement in 2018 redefined essential tremor as an isolated action tremor present in bilateral upper extremities for at least three years. Tremor may also be present in other locations, commonly the neck or the vocal cords. Patients with additional neurologic symptoms are now categorized as "essential tremor plus." Additional clinical features associated with the condition include but are not limited to cognitive impairment, psychiatric disorders, and hearing loss. When treatment is needed, propranolol and primidone are considered first line treatments. Patients who are severely affected are often offered deep brain stimulation. Although the ventral intermediate nucleus of the thalamus is the traditional surgical target, the caudal zona incerta is also being studied as a possible superior alternative. Magnetic resonance imaging guided high intensity focused ultrasound is a newer surgical alternative that may be ideal for patients with substantial medical comorbidities. Current research explores novel oral treatments, chemodenervation, and noninvasive neuromodulation for treatment of essential tremor., Competing Interests: Competing interests: I have read and understood the BMJ policy on declaration of interests and declare the following interests: none., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Introduction: To explore the prevalence, clinical course and associated features of lower limb tremor in the most common tremor syndromes., Methods: This retrospective chart review studied lower limb tremor patients as defined by a Tremor Rating Scale score ≥1 in either lower limb. We compared and correlated their characteristics, also comparing them with patients without lower limb tremor., Results: Of the 283 patients with lower limb tremor (58.3% males, age: 65.0 ± 16.0 years, tremor duration: 25.6 ± 17.9 years), 255 patients within six tremor syndrome groups were included in the final analysis. Prevalence of patients with lower limb tremor (either rest, postural or kinetic) was lowest (28.6%) in the essential tremor (ET) group and highest (69%) in the parkinsonian tremor (PT) group. Lower limb tremor score was higher in patients classified as having intention tremor (IT) compared to ET and dystonic tremor (DT). Total tremor score was highest in IT and lowest in ET. We found a positive correlation between total lower limb tremor score and total tremor score in most groups. In addition, there was a positive correlation between lower limb tremor score and upper limb tremor score. Finally, compared to patients without lower limb tremor, all diagnostic groups with lower limb tremor, with the exception of functional tremor (FT), had worse total tremor score; and disease duration was longer in ET-plus, DT and PT patients with lower limb tremor compared to those without., Conclusions: Lower limb tremor is less commonly observed in ET than other tremor syndromes, is a marker of symptom severity of the underlying disease condition in all tremor syndromes except FT, and reflects longer disease duration in ET-plus, DT and PT., (Copyright © 2019 Elsevier Ltd. All rights reserved.)