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Myotonia and flaccid dysarthria in patients with adult onset myotonic dystrophy
  1. B J M de Swart1,
  2. B G M van Engelen1,
  3. J P B M van de Kerkhof2,
  4. B A M Maassen3
  1. 1Neuromuscular Centre and Institute of Neurology, University Medical Centre Nijmegen, Nijmegen, Netherlands
  2. 2Department of Speech and Language, University of Nijmegen
  3. 3Medical Psychology and Child Neurology Centre, University Medical Centre Nijmegen
  1. Correspondence to:
 B J M de Swart
 Neuromuscular Centre Nijmegen, Institute of Neurology, University Medical Centre Nijmegen, PO Box 9101, 6500 HB Nijmegen, Netherlands; b.deswartneuro.umcn.nl

Abstract

Background: Myotonia and weakness are the most important components of dysarthric speech in myotonic dystrophy.

Objective: To specify and quantify possible defects in speech execution in patients with adult onset myotonic dystrophy.

Methods: Studies on speech production were done on 30 mildly affected patients with myotonic dystrophy. Special attention was paid to myotonia. Because muscle activity can result in a decrease of myotonia, speech characteristics were measured before and after warm up. The possibility that warming up causes increased weakness was also assessed.

Results: As with other motor skills, a warm up effect was found in speech production, resulting in an increase in repetition rate and a decrease in variability of repetition rate. Signs of fatigue did not occur.

Conclusions: Warming up is valuable for patients with myotonic dystrophy in reducing the influence of myotonia on speech production.

  • MRR, maximum repetition rate
  • MSP, maximum sound prolongation
  • myotonic dystrophy
  • dysarthria
  • warm up

https://doi.org/10.1136/jnnp.2003.032151

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    Footnotes

    • Competing interests: none declared