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Reclassification of patients with tremor syndrome and comparisons of essential tremor and essential tremor-plus patients

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Abstract

Background

The new essential tremor (ET)-plus nomenclature was proposed by the 2018 Tremor Consensus Criteria. However, few studies have adopted this usage and the clinical differences between ET and ET-plus remains unclear. To address this issue, we reclassified and compared the characteristics of ET and ET-plus patients in a large Chinese tremor cohort.

Methods

In this cross-sectional observational study, 766 patients originally diagnosed with ET underwent neurological examination. Scale ratings were used to evaluate motor and non-motor symptoms, and quality of life (QoL). We then reclassified the ET cohort and compared demographic and clinical characteristics between ET and ET-plus patients. A logistic regression analysis was used to explore whether the presence of neurological soft signs in ET-plus was associated with tremor severity or QoL.

Results

Among 665 clinically confirmed ET syndrome patients, 274 were ET and 391 were ET-plus. The most prevalent neurological soft sign was resting tremor. ET-plus patients were older, had older age at onset and longer disease duration. ET-plus patients recorded higher scores in tremor severity evaluations and lower in cognitive evaluations, whereas a higher proportion of patients presented with depression or anxiety symptoms. Resting tremor and questionable cerebellar signs were associated with tremor severity. Cognitive impairment was associated with worse QoL.

Conclusions

ET-plus patients were older, had longer disease durations, worse tremor manifestations, and more distinct non-motor symptoms. Certain additional soft signs of ET-plus were associated with tremor severity or worse QoL. ET-plus patients may include advanced ET patients with additional neurological soft signs presenting along with disease progression.

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Data availability

The data used to support the findings of this study cannot be shared at this time because it is utilised in an ongoing study.

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Acknowledgements

The authors would like to acknowledge Professors Dong Zhou, Li He, and Bo Wu for their support in the entire study. We would also like to thank Dr. Ling Wang for collecting the clinical data and express our sincere appreciation to the families who participated in this study.

Funding

The study was supported by the National Natural Science Foundation of China (81801272), the Sichuan Science and Technology Program (2018FZ0029 and 2018HH0077), and The Fundamental Research Funds for the Central Universities (2018SCU12029).

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Authors and Affiliations

  1. Department of Neurology, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China

    Jiaxin Peng, Nannan Li, Junying Li, Liren Duan, Chaolan Chen, Yan Zeng, Jing Xi, Yi Jiang & Rong Peng

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  1. Jiaxin Peng
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  2. Nannan Li
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Correspondence to Rong Peng.

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Conflicts of interest

The authors declared no conflicts of interest.

Ethical standards

The study conforms with the premises of the Declaration of Helsinki and was approved by the ethics committee of West China Hospital of Sichuan University.

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Peng, J., Li, N., Li, J. et al. Reclassification of patients with tremor syndrome and comparisons of essential tremor and essential tremor-plus patients. J Neurol 269, 3653–3662 (2022). https://doi.org/10.1007/s00415-022-10985-4

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  • DOI: https://doi.org/10.1007/s00415-022-10985-4

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