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Tremor in Primary Monogenic Dystonia

Sanjay Pandey1, Sonali Bhattad2, Shreya Dinesh2

  • 1Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, Academic Block, Room No 501, New Delhi, 110002, India. sanjaysgpgi2002@yahoo.co.in.

Current Neurology and Neuroscience Reports
|July 15, 2021
PubMed
Abstract

Insights

Tremor is a common dystonia symptom, but prevalence varies. This review found tremor in nine primary monogenic dystonia genes, with DYT-HPCA and DYT-ANO3 showing high prevalence in isolated dystonia.

Area of Science:

  • Neurology
  • Genetics
  • Movement Disorders

Background:

  • Tremor is a key feature of dystonia, with reported prevalence varying widely (14-86.67%).
  • This variability may stem from different dystonia types studied.
  • Understanding tremor in primary monogenic dystonia is crucial for diagnosis and treatment.

Purpose of the Study:

  • To review research on tremor prevalence in primary monogenic dystonia.
  • To identify specific genes associated with tremor in dystonia.

Main Methods:

  • Searched the MDS gene database for research articles on tremor in primary monogenic dystonia.
  • Analyzed reported frequencies of tremor across different dystonia genes.

Main Results:

  • Tremor was identified in nine primary monogenic dystonia genes (DYT-HPCA, DYT-ANO3, DYT-KCTD17, DYT-THAP1, DYT-PRKRA, DYT-GNAL, DYT-TOR1A, DYT-KMT2B, DYT-SGCE).
  • DYT-HPCA and DYT-ANO3 showed the highest tremor prevalence in isolated dystonia.
  • DYT-SLC6A3 exhibited the highest tremor prevalence in dystonia parkinsonism.

Conclusions:

  • Specific genes are frequently associated with tremor in primary monogenic dystonia.
  • Gene identification aids in understanding tremor's role in dystonia phenotypes.
  • Further research can refine the understanding of tremor in genetic dystonia subtypes.
Keywords:
DystoniaGeneParkinsonismTremor

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