Background: Patients with essential tremor were initially considered to have isolated tremor, but additional motor and non-motor features have been increasingly recognized The term “essential tremor plus” was adopted by the Task Force on Tremor of the International Parkinson and Movement Disorder Society to describe essential tremor patients with additional neurologic signs Objectives: To characterize essential tremor patients and their phenotypes in a movement disorders clinic population in the context of the new tremor classification Methods: Demographic, clinical, historical, treatment, and diagnostic data were retrospectively collected on 300 patients diagnosed by movement disorder experts with essential tremor Patients were classified as having essential tremor, essential tremor plus, or essential tremor-Parkinson’s disease combination, and features between these groups were compared Results: Of the 300 patients, 207% were classified as isolated essential tremor, 533% as essential tremor plus, and 260% as essential tremor-Parkinson’s disease There was no significant difference in the duration of tremor symptoms Essential tremor plus patients were more likely to have dystonia, tandem gait abnormalities, head tremor and greater tremor severity Essential tremor-Parkinson’s disease patients were more likely to have RBD symptoms There was no significant difference in cognitive impairment between essential tremor plus and essential tremor-Parkinson’s disease patients Conclusions: Additional motor and non-motor features, including parkinsonism, are common in patients with essential tremor Further studies are needed to clarify essential tremor phenotypes and to provide insights into possible subtypes Highlights: 300 patients with essential tremor from a movement disorders clinic were re-classified based on the Movement Disorder Society Consensus Statement on the Classification of Tremors Additional motor and non-motor features, including parkinsonism, were common, and only 207% of patients remained classified as isolated essential tremor

/pdf/phenotypic-features-of-isolated-essential-tremor-essential-538o1dof3s.pdf

Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson's Disease in a Movement Disorders Clinic

Features of "ET plus" correlate with age and tremor duration: "ET plus" may be a disease stage rather than a subtype of essential tremor.

The Clinical Manual

Consensus Paper: Ataxic Gait

Essential tremor (ET) is a common and heterogeneous disorder characterized by postural/kinetic tremor of the upper limbs and other body segments and by non‐motor symptoms, including cognitive and psychiatric abnormalities. Only a limited number of longitudinal studies have comprehensively and simultaneously investigated motor and non‐motor symptom progression in ET. Possible soft signs that configure the ET‐plus diagnosis are also under‐investigated in follow‐up studies. We aimed to longitudinally investigate the progression of ET manifestations by means of clinical and neurophysiological evaluation.

https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/ene.15650

Longitudinal study of clinical and neurophysiological features in essential tremor

Background: Essential tremor (ET) encompasses a variety of features, including tremor, cognitive dysfunction, and gait and balance impairments. Gait and balance impairments in ET are often mild, but they can be severe and are, in some cases, associated with functional sequelae in terms of increased fall risk and reduced balance confidence. Previous research on gait and balance in ET has been limited to cross-sectional comparisons. There have been no longitudinal studies or prospective studies. As such, our understanding of natural history and possible predictors of declines in ET-related gait and balance impairments is incomplete. Objectives: We (1) present natural history data on the change in gait and balance measures over time, (2) provide estimates of annual rate of change in each gait and balance metric, and (3) examine the relationship between baseline clinical predictors and changes in gait and balance over time. Methods: 149 ET participants (mean age 78.7 years), enrolled in a prospective, longitudinal, clinical-pathological study, underwent an extensive evaluation of cognition, tremor, and gait and balance at three distinct intervals performed every 18 months. Gait and balance measures included a combination of performance-based tests (e.g., tandem gait, tandem stance) and self-reported assessments (e.g., number of falls, use of a walking aid). Results: Between the baseline and final assessments, numerous balance and gait measures showed evidence of decline and annual rates of change were quantified for each. We examined the predictive utility of clinical variables at baseline for five gait and balance outcomes, with global cognition and executive function standing out as the most consistent predictors. Conclusions: We present a much-needed look into the course of disease for elderly patients with ET, focusing on changes observed in gait and balance and the predictors of these changes. These results also add another dimension to the relevance of cognitive impairment observed in ET; such impairment can now be viewed as predictive of poorer gait and balance over time in ET. These findings are a useful tool for clinicians, patients, and their families to better understand and plan for changing disease-features over time.

/pdf/prospective-longitudinal-study-of-gait-and-balance-in-a-3c56if5mts.pdf

Prospective Longitudinal Study of Gait and Balance in a Cohort of Elderly Essential Tremor Patients.

Depressive symptoms predict memory decline in Essential Tremor.

Rate of progression from mild cognitive impairment to dementia in an essential tremor cohort: A prospective, longitudinal study.

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Papers

Prospective Longitudinal Study of Gait and Balance in a Cohort of Elderly Essential Tremor Patients.

Depressive symptoms predict memory decline in Essential Tremor.

Rate of progression from mild cognitive impairment to dementia in an essential tremor cohort: A prospective, longitudinal study.