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Updated: Jan 7, 2026

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The Cerebellar Cognitive Affective Syndrome in Essential Tremor Plus.

R Erro1,2, M Russo3, R Bisogno3

  • 1Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", Neuroscience Section, University of Salerno, Baronissi, SA, Italy. rerro@unisa.it.

Cerebellum (London, England)
|December 6, 2025
PubMed
Summary

Essential Tremor Plus (ET-plus) patients exhibit significant cognitive impairment, potentially linked to cerebellar dysfunction. These deficits impact daily living, suggesting ET-plus may be a distinct neurological entity.

Keywords:
CerebellumCognitive impairmentDementiaQuality of life

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Area of Science:

  • Neurology
  • Neuropsychology

Background:

  • Essential Tremor (ET) is now classified into pure (pET) and ET-plus forms.
  • Cognitive dysfunction in ET may relate to Cerebellar Cognitive Affective Syndrome (CCAS), but evidence in ET-plus is lacking.

Purpose of the Study:

  • To evaluate CCAS in ET-plus patients.
  • To explore motor-cognitive associations in ET-plus.
  • To compare ET-plus cognitive profiles with pET.

Main Methods:

  • CCAS-scale (CCAS-S) assessment in 39 ET-plus patients and 19 healthy controls (HC).
  • Evaluation using the Essential Tremor Rating Scale and Scale for the Assessment and Rating of Ataxia.
  • Recording of soft signs and comparison with published pET data.

Main Results:

  • ET-plus patients showed poorer CCAS-S performance than HC, with 69.4% diagnosed with definite CCAS.
  • Cognitive performance correlated with slowing, not other clinical data.
  • Definite CCAS rates were similar in pET and ET-plus, but cognitive profiles differed.
  • Poorer CCAS-S scores negatively impacted daily living beyond tremor severity.

Conclusions:

  • ET-plus presents pervasive cognitive impairment, likely due to cerebellar dysfunction.
  • Cognitive deficits associated with slowing and differing profiles between ET-plus and pET suggest broader brain involvement.
  • ET-plus and pET may represent distinct neurological conditions.