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Tremor in Primary Monogenic Dystonia

  • Movement Disorders (T. Simuni, Section Editor)
  • Published:
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Abstract

Purpose of Review

Tremor is an important phenotypic feature of dystonia with wide variability in the reported prevalence ranging from 14 to 86.67%. This variability may be due to the types of dystonia patients reported in different studies. This article reviews research articles reporting tremor in primary monogenic dystonia.

Recent Findings

We searched the MDS gene data and selected all research articles reporting tremor in primary monogenic dystonia. Tremor was reported in nine dystonia genes, namely DYT-HPCA, DYT-ANO3, DYT-KCTD17, DYT-THAP1, DYT-PRKRA, DYT-GNAL, DYT-TOR1A, DYT-KMT2B, and DYT-SGCE in the descending order of its frequency. HPCA gene mutation is rare, but all reported patients had tremor. Similarly, tremor was reported in eight genes associated with dystonia parkinsonism, namely DYT-SLC6A3, DYT-TH, DYT-SPR, DYT-PTS, DYT-GCH1, DYT-TAF1, DYT-QDPR, and DYT-SCL30A10 in the descending order of its prevalence.

Summary

DYT-HPCA and DYT-ANO3 gene showed the highest prevalence of tremor in isolated dystonia, and DYT-SLC6A3 has the highest prevalence of tremor in combined dystonia.

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Papers of particular interest, published recently, have been highlighted as: •• Of major importance

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Acknowledgements

This “Point of view” article is not funded by any organization.

Funding

The Junior research fellowship for Shreya Dinesh (co-author) was funded by a grant from the Department of Biotechnology from project # BT/PR26428/Med/12/783/2018.

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Authors and Affiliations

  1. Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, Academic Block, Room No 501, New Delhi, 110002, India

    Sanjay Pandey, Sonali Bhattad & Shreya Dinesh

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  1. Sanjay Pandey
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  2. Sonali Bhattad
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  3. Shreya Dinesh
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Contributions

Sanjay Pandey, Sonali Bhattad, and Shreya Dinesh contributed in manuscript preparation by writing the first draft, review, and critique.

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Correspondence to Sanjay Pandey.

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Sanjay Pandey, Sonali Bhattad, and Shreya Dinesh each declare no potential conflicts of interest.

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Pandey, S., Bhattad, S. & Dinesh, S. Tremor in Primary Monogenic Dystonia. Curr Neurol Neurosci Rep 21, 48 (2021). https://doi.org/10.1007/s11910-021-01135-w

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