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Essential tremor

Nature Reviews Disease Primers volume 7, Article number: 83 (2021) Cite this article

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Abstract

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.

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Fig. 1: Prevalence of essential tremor based on studies from 2001–2021.
Fig. 2: Risk and aetiology of essential tremor.
Fig. 3: Key pathways involved in the pathophysiology of essential tremor.
Fig. 4: Management of essential tremor.

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Acknowledgements

The authors gratefully acknowledge E. Louis (University of Texas Southwestern Medical Center, USA) for his input on the pathophysiology section. They acknowledge support of the National Medical Research Council under its Open Fund Large Collaborative Grant (MOH-OFLCG18May-0002 and STAR award to E.K.-T.). T.W. acknowledges the support of the National Medical Research Council (OFYIRG20nov-0032).

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Authors and Affiliations

  1. Department of Research, National Neuroscience Institute, Singapore, Singapore

    Thomas Welton & Eng-King Tan

  2. Neuroscience Academic Clinical Programme, Duke-NUS Medical School, Singapore, Singapore

    Thomas Welton & Eng-King Tan

  3. Movement Disorders Unit, Neurology Service, Internal Medicine Department, University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil

    Francisco Cardoso

  4. Division of Neurology, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa

    Jonathan A. Carr

  5. Department of Diagnostic Radiology, Singapore General Hospital, Singapore, Singapore

    Ling-Ling Chan

  6. Radiological Sciences Academic Clinical Programme and Neuroscience & Behavioral Disorders Signature Research Programme, Duke-NUS Medical School, Singapore, Singapore

    Ling-Ling Chan

  7. Department of Neurology, Christian Albrechts University, Kiel, Germany

    Günther Deuschl

  8. Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, USA

    Joseph Jankovic

  9. Department of Neurology, National Neuroscience Institute, Singapore, Singapore

    Eng-King Tan

Authors
  1. Thomas Welton
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  2. Francisco Cardoso
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  3. Jonathan A. Carr
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  4. Ling-Ling Chan
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  5. Günther Deuschl
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  6. Joseph Jankovic
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Contributions

Introduction (E.-K.T., T.W., J.A.C., L.-L.C., G.D. and J.J.); Epidemiology (E.-K.T., T.W. and J.A.C.); Mechanisms/pathophysiology (E.-K.T., T.W. and G.D.); Diagnosis, screening and prevention (E.-K.T., T.W., F.C. and J.J.); Management (T.W.); Quality of life (E.-K.T. and T.W.); Outlook (E.-K.T., T.W., F.C., J.A.C., L.-L.C., G.D. and J.J.); Overview of Primer (E.-K.T.).

Corresponding author

Correspondence to Eng-King Tan.

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Nature Reviews Disease Primers thanks Alfonso Fasano, Félix Jiménez-Jiménez, Sheng-Han Kuo and the other, anonymous, reviewer(s) for their contribution to the peer review of this work.

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Glossary

Action tremor

Tremor that occurs with the voluntary movement of a muscle.

Postural tremor

Tremor that occurs when a person maintains a position against gravity.

Kinetic tremor

Tremor that occurs with movement.

ET plus

Essential tremor (ET) with other neurological signs.

Familial ET

Essential tremor (ET) in a patient with a family history of ET, usually consistent with autosomal dominant transmission.

Sporadic ET

Essential tremor (ET) in a patient without an underlying cause or family history (also known as idiopathic ET).

Sensory trick

A motor or sensory manoeuvre that temporarily alleviates dystonia.

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Welton, T., Cardoso, F., Carr, J.A. et al. Essential tremor. Nat Rev Dis Primers 7, 83 (2021). https://doi.org/10.1038/s41572-021-00314-w

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