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Ptosis aggravates dysphagia in oculopharyngeal muscular dystrophy
  1. B J M de Swart1,
  2. B M van der Sluijs1,
  3. A M C Vos3,
  4. J G Kalf2,
  5. S Knuijt2,
  6. J R M Cruysberg3,
  7. B G M van Engelen1
  1. 1Neuromuscular Centre Nijmegen, Institute of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  2. 2Department of Speech Pathology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  3. 3Department of Ophthalmology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  1. Correspondence to:
 Bert de Swart
 304 Neuromuscular Centre Nijmegen, Institute of Neurology, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands;b.deswart{at}neuro.umcn.nl

Abstract

Background: Ptosis and dysphagia are important features in oculopharyngeal muscular dystrophy (OPMD).

Objective: Retroflexion of the head is a well known compensatory mechanism for ptosis, but generally retroflexion has a negative effect on swallowing. We hypothesised that severity of ptosis is related to degree of retroflexion and that this compensation is responsible for deteriorating dysphagia.

Methods: Nine OPMD patients were examined in the conditions “head position adapted to ptosis” and “head position slightly flexed”. Ptosis was quantified by photogrammetry and retroflexion of the head by digital photographs. The severity of dysphagia was measured using visual analogue scales (VAS) and by calculating swallowing volumes and oropharyngeal swallow efficiency (OPSE) based on videofluoroscopy.

Results: Statistical analyses show a significant relationship between ptosis and degree of retroflexion. The degree of retroflexion of the head correlated significantly with VAS scores and with the maximum swallowing volume. The slightly flexed head position significantly improved VAS scores as well as swallowing volumes and OPSE.

Conclusion: In OPMD patients, ptosis significantly correlates with retroflexion of the head, which has a negative effect on swallowing. Subjective and objective reduction of swallowing problems was found when patients were instructed to eat and drink with a slightly flexed head position.

  • OPMD, oculopharyngeal muscular dystrophy
  • OPSE, oropharyngeal swallow efficiency
  • VAS, visual analogue scale
  • deglutition disorders
  • dysphagia
  • oculopharyngeal muscular dystrophy
  • OPMD
  • ptosis

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Footnotes

  • Competing interests: none declared

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