The relevance and accuracy of functional electrophysiological tests in canalopathies and myotonia

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The team at the Tampere Reference Centre for Neuromuscular Diseases (Finland) examined the results of functional electrophysiological tests in 27 patients with canalopathy, myotonic dystrophy type 1 (DM1) or type 2 (DM2) :

  • the most frequent symptoms were muscle weakness, myalgias, cramps and muscle stiffness,
  • the median age at the time of functional electrophysiological testing was 43.2 years,
  • 59.3% had functional tests (short effort, long effort, cold test and short effort) compatible with Fournier’s scheme,
  • 33.3% had only one abnormal functional test,
  • 18.5% had no electrophysiological abnormality (2 had DM2 and 3 had heterozygous SCN4A abnormalities).

The authors conclude that more precise and sensitive neurophysiological tests are needed to detect muscle channelopathies.

 

A retrospective study of accuracy and usefulness of electrophysiological exercise tests. Periviita V, Jokela M, Palmio et al. J Neurol. 2023 Dec 6.

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