Early‐ and late‐onset essential tremor patients represent clinically distinct subgroups

Hopfner, Franziska; Ahlf, Anjuli; Lorenz, Delia; Klebe, Stephan; Zeuner, Kirsten E.; Kuhlenbäumer, Gregor; Deuschl, Günther

doi:10.1002/mds.26708

Cited by 69 publications

(77 citation statements)

References 37 publications

(87 reference statements)

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“…Tremor onset after the age of 65 years is associated with an increased risk of dementia, a different natural history than those with tremor onset earlier in life. 18 On the other hand, family history and alcohol responsiveness may be more common in early-onset “ET”, 19 and may be associated with other tremor syndromes to a similar or greater extent. Yet they continue to be used as supportive of the diagnosis of ET.…”

Section: The Pitfalls In the Diagnosis Of Etmentioning

confidence: 99%

Essential pitfalls in “essential” tremor

et al. 2017

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While essential tremor has been considered the most common movement disorder, it has largely remained a diagnosis of exclusion: many tremor and non-tremor features must be absent for the clinical diagnosis to stand. The clinical features of “essential tremor” overlap with or may be part of other tremor disorders and, not surprisingly, this prevalent familial disorder has remained without a gene identified, without a consistent natural history, and without an acceptable pathology or pathophysiologic underpinning. The collective evidence suggests that under the rubric of essential tremor there exists multiple unique diseases, some of which represent cerebellar dysfunction, but for which there is no intrinsic “essence” other than a common oscillatory behavior on posture and action. One approach may be to use the term “essential tremor” only as a transitional node in the deep phenotyping of tremor disorders based on historical, phenomenological, and neurophysiological features, to facilitate its etiologic diagnosis or serve for future gene- and biomarker-discovery efforts. This approach deemphasizes essential tremor as a diagnostic entity and facilitates the understanding of the underlying disorders in order to develop biologically tailored diagnostic and therapeutic strategies.

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Section: The Pitfalls In the Diagnosis Of Etmentioning

confidence: 99%

Essential pitfalls in “essential” tremor

et al. 2017

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“…Second, even if it were conceptually of merit, the cutoff point for such an agerelated tremor is arbitrary [68]. Even the authors who suggested this term have defined "late-onset" differently in various papers (e.g., defining it as ≥46 years in another paper) [69]. Finally, postmortem studies of humans suggest that the same degenerative pathology is found in both younger onset and older onset ET cases to the same extent [70].…”

Section: Additional Biological Considerations: Aging and Etmentioning

confidence: 99%

The Roles of Age and Aging in Essential Tremor: An Epidemiological Perspective

Louis

2019

Neuroepidemiology

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Background: With an aging population, it behooves physicians and scientists to gain an understanding of the intersections between aging and human disease. Here, the author reviews the intersections between age, aging, and essential tremor (ET), particularly as these relate to disease epidemiology. Summary: The prevalence and incidence of ET both increase with age, and the former, in an exponential fashion with advanced aging. There is evidence that age may independently drive several features of the natural history of ET (e.g., the increase in tremor severity with time, the appearance of head tremor with time). Epidemiological studies clearly show that ET is associated with several conditions of advanced age, including Parkinson’s disease, mild cognitive impairment and dementia. Aside from age, age of onset has several important clinical correlates – familial forms of ET are more likely to have younger ages of onset, and older ages of onset are associated with more rapid disease progression. Key Messages: Overall, there are several important intersection points between age, aging, and ET. Potential biological explanations for these observations are discussed. Further knowledge about the basis for these observations will enhance our understanding of this disease and inform our care of patients.

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“…Second, in terms of pathophysiologies, Lewy bodies in locus ceruleus or torpedos in Purkinje cells have been found in post‐mortem study, and three hypotheses, the olivary pacemaker hypothesis, GABA hypothesis, and the cerebellar degeneration hypothesis, may be involved . And further, patients having cranial tremor or late onset age are present distinct clinical features or neuroimaging changes . Moreover, the links between these factors above are still being studied.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of patients with essential tremor or essential tremor plus

et al. 2020

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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.

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Early‐ and late‐onset essential tremor patients represent clinically distinct subgroups

Cited by 69 publications

References 37 publications

Essential pitfalls in “essential” tremor

Essential pitfalls in “essential” tremor

The Roles of Age and Aging in Essential Tremor: An Epidemiological Perspective

Clinical characteristics of patients with essential tremor or essential tremor plus

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