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Essential tremor—a neurodegenerative disorder associated with cognitive defects?

Nature Reviews Neurology volume 7, pages 273–282 (2011)Cite this article

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Abstract

In the past decade, the hypothesis that essential tremor is a monosymptomatic tremorogenic disorder has been questioned. New clinical, neuroimaging, electrophysiological and pathological studies indicate that essential tremor is associated with subtle neurological deficits and could be considered a slowly progressive neurodegenerative disorder. The aim of this Review is to describe the nonmotor neurological symptoms that are commonly associated with essential tremor, and highlight the cognitive deficits associated with this condition. Several clinical studies have demonstrated that essential tremor is associated with mild deficits in attention, executive functions, memory and, possibly, other cognitive processes. Population-based surveys have confirmed that dysfunction in these cognitive domains affects patients both with severe and mild essential tremor. Clinical studies have also indicated that cognitive deficits associated with essential tremor are progressive and that patients with this condition have an increased risk of dementia. Mood and cognitive deficits commonly observed in patients with essential tremor are similar to symptoms of cerebellar cognitive affective syndrome. Further evidence is required from prospective studies to support the interpretation that essential tremor is a slowly progressive neurodegenerative disorder.

Key Points

  • Essential tremor was originally considered to be a monosymptomatic movement disorder without known CNS pathology

  • New research suggests that essential tremor is a slowly progressive neurodegenerative disorder predominantly associated with cerebellar pathology

  • Research indicates that patients with essential tremor commonly experience neurological abnormalities such as bradykinesia, ocular movement anomalies, deficits in hand–eye coordination, mild dysarthria, gait ataxia, and probably olfactory and hearing deficits

  • Essential tremor is associated with mild cognitive impairment and mood disorders including depression and anxiety, and also with an increased risk of dementia

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Figure 1: 37-MMSE scores in patients with essential tremor and controls in the Neurological Disorders in Central Spain (NEDICES) cohort.65

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Acknowledgements

Thanks to Dr J. Benito-León for his comments on the manuscript, V. Puertas-Martin for her advice on the neuropsychological data and to J. Healey for correcting many awkward English expressions in the manuscript. L. Barclay, freelance writer and reviewer, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.

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  1. Department of Neurology, University Hospital “Doce de Octubre”, Avenida de Córdoba s/n, 28041, Madrid, Spain

    Félix Bermejo-Pareja

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  1. Félix Bermejo-Pareja
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Supplementary information

Supplementary Table 1

Main deficits in cognitive tests in patients with essential tremor* (DOC 121 kb)

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Bermejo-Pareja, F. Essential tremor—a neurodegenerative disorder associated with cognitive defects?. Nat Rev Neurol 7, 273–282 (2011). https://doi.org/10.1038/nrneurol.2011.44

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