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The relation between daytime sleepiness, fatigue, and reduced motivation in patients with adult onset myotonic dystrophy
  1. Netherlands Fatigue Research Group1,
  2. S van der Werf1,
  3. J Kalkman1,
  4. G Bleijenberg1,
  5. B van Engelen2,
  6. M Schillings3,
  7. M Zwarts3
  1. 1Department of Medical Psychology, University Medical Centre Nijmegen, PO box 9101, 6500 HB Nijmegen, Netherlands
  2. 2Neuromuscular Centre Nijmegen, Institute of Neurology, University Medical Centre Nijmegen
  3. 3Department of Clinical Neurophysiology, University Medical Centre Nijmegen
  1. Correspondence to:
 Dr S P van der Werf;
 s.vanderwerf{at}cukz.umcn.nl

https://doi.org/10.1136/jnnp.74.1.138

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    Daytime sleepiness, apathy, and lack of motivation are established clinical manifestations of myotonic dystrophy.1,2 A recent study showed that modafinil reduced daytime sleepiness and average sleep latency in a group of nine patients with myotonic dystrophy.3 This finding suggests that daytime sleepiness in patients with myotonic dystrophy and without obstructive sleep apnoea might be central in origin. A magnetic resonance imaging study indeed found evidence for a possible association between cerebral abnormalities in myotonic dystrophy and excessive daytime sleepiness.4 Although several studies have measured levels of fatigue with validated questionnaires in different neurological patient populations,5,6 fatigue questionnaires have not yet been related to the symptoms of daytime sleepiness in myotonic dystrophy. With the results of the modafinil study mentioned above in mind, our goal was to test the relations between excessive daytime sleepiness, experienced fatigue, and reduced motivation.

    Methods

    Patients

    The study was conducted at the outpatient clinic of the Neuromuscular Centre Nijmegen, based at the Institute of Neurology of the University Medical Centre Nijmegen in the Netherlands. Consecutive ambulant patients with a genetically confirmed diagnosis of (adult onset) myotonic dystrophy and an expanded CTG repeat on chromosome 19q13.3 (DM1) were invited to take part. Fatigue was not a criterion …

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    Footnotes

    • Competing interests: none declared