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Measuring Myotonia: Normative Values and Comparison with Myotonic Dystrophy Type 1.

Andrea Sipos1, Milán Árvai1, Dávid Varga1

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Summary

This study establishes normative values for myotonia relaxation tests in Hungary. Patients with myotonic dystrophy type 1 (DM1) showed significant differences compared to healthy individuals, highlighting the tests

Keywords:
dynamometrymyotoniamyotonic dystrophynine-hole peg testnormative values

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Area of Science:

  • Neurology
  • Clinical Neuroscience
  • Rehabilitation Medicine

Background:

  • Myotonia, a rare neuromuscular disorder, is characterized by delayed muscle relaxation.
  • Establishing population-specific normative data is crucial for accurate clinical assessment.
  • Myotonic Dystrophy Type 1 (DM1) is a primary genetic cause of myotonia.

Purpose of the Study:

  • To determine normative values for myotonia-related clinical tests in the Hungarian population.
  • To compare these normative values with those of patients diagnosed with myotonic dystrophy type 1 (DM1).
  • To evaluate the influence of age and sex on muscle relaxation and strength in healthy individuals.

Main Methods:

  • Conducted relaxation tests (eye, tongue, palm openings), handgrip strength, and nine-hole peg test.
  • Recruited 139 healthy Hungarian individuals and 31 patients with DM1.
  • Analyzed data for age and sex-related differences in controls and compared controls to DM1 patients.

Main Results:

  • No significant age-related decline observed in handgrip strength or relaxation tests (p < 0.05).
  • Significant differences (p < 0.05) were found in all tested parameters between healthy controls and DM1 patients.
  • Healthy males exhibited stronger handgrip and slower relaxation times compared to females; these sex differences were absent in DM1 patients.

Conclusions:

  • Normative values for myotonia relaxation tests across various age groups in the Hungarian population were successfully established.
  • The study supports using population averages rather than age-specific groups for relaxation test evaluations in myotonia.
  • These findings provide valuable reference data for the clinical evaluation and diagnosis of myotonia, particularly DM1.