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Non-motor features of essential tremor with midline distribution

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Abstract

Background

Midline essential tremor (Mid-ET) is a distinctive group of essential tremor (ET) in which tremor affects the neck, jaw, tongue, and/or voice. For long, it has been considered as an ultimate stage of the disease and a marker of its severity. However, recent studies pointed its complexity in terms of non-motor presentation. Thus, we aimed to investigate the non-motor signs (NMS) in Mid-ET.

Design

We conducted a cross-sectional study in a tertiary neurology referral center including ET patients classified into two groups based on the presence or not of midline tremor (Mid-ET vs. No-Mid-ET). We assessed NMS using the non-motor severity scale (NMSS), a large battery of cognitive tests, clinical and electrophysiological study of the autonomic nervous system along with the evaluation of sleep disturbances.

Results

A total of 163 patients were included: Mid-ET (n = 79) and No-Mid-ET (n = 84) matched in gender and age of onset. Mid-ET patients had higher proportion of late-onset ET (> 60 years old, p = 0.002) and more extrapyramidal signs (p = 0.005). For NMS, Mid-ET was marked with cognitive dysfunction (p = 0.008). The hallmarks of the neuropsychiatric profile of Mid-ET were executive dysfunction (p = 0.004), attention problems (p < 0.000), episodic memory impairment (p = 0.003), and greater depression (p = 0.010). The presence of RBD was a trait of Mid-ET (p = 0.039). In both Mid-ET and No-Mid-ET phenotypes, clinical and neurophysiological dysautonomia correlated with cognitive dysfunction.

Conclusion

Mid-ET patients had greater cognitive dysfunction, depression, RBD, higher proportion of late-onset ET, and more extrapyramidal signs. Taken all together, these findings could provide a redesigned insight into the underlying physiopathology of Mid-ET indicative of a greater cerebellar dysfunction.

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Acknowledgements

We thank all patients who gave their consent and participated in the present study.

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Authors and Affiliations

  1. Neurology Department, Razi University Hospital, 1 rue des orangers Manouba, LR18SP032010, Tunis, Tunisia

    Arwa Rekik, Amina Nasri, Saloua Mrabet, Alya Gharbi, Amira Souissi, Amina Gargouri, Imen Kacem & Riadh Gouider

  2. Faculty of Medicine of Tunis, University of Tunis El Manar, 15, Rue Djebel Akhdhar, La Rabta, 1007, Tunis, Tunisia

    Amina Nasri, Saloua Mrabet, Alya Gharbi, Amira Souissi, Amina Gargouri, Imen Kacem & Riadh Gouider

  3. Clinical Investigation Center (CIC) “Neurosciences and Mental Health”, Razi University Hospital, 1 rue des orangers Manouba, 2010, Tunis, Tunisia

    Amina Nasri, Saloua Mrabet, Alya Gharbi, Amira Souissi, Amina Gargouri, Imen Kacem & Riadh Gouider

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  1. Arwa Rekik
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Correspondence to Riadh Gouider.

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The ethical committee of Razi Hospital approved this process, and all patients were de-identified.

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Rekik, A., Nasri, A., Mrabet, S. et al. Non-motor features of essential tremor with midline distribution. Neurol Sci 43, 5917–5925 (2022). https://doi.org/10.1007/s10072-022-06262-x

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