Retinal changes in myotonic dystrophy. Clinical and follow-up evaluation

Retina. 1993;13(2):129-35.


Retinal changes in myotonic dystrophy include butterfly-shaped pigmentary changes in the macula, reticular pigmentary retinal changes in the midperiphery, and peripheral atrophic polygonal-shaped changes. To assess the prevalence and progressiveness of these changes in a large series, 49 patients with myotonic dystrophy were examined. Of the eyes studied, 26 (26.6%) had butterfly-shaped macular pigmentary changes, 24 (24.5%) had reticular pigmentary retinal changes, and 43 (43.9%) had peripheral atrophic polygonal-shaped changes. Ten patients (18 eyes) had two different types at once. Visual acuity was not severely affected. Electroretinographic examination showed diminished a and b wave amplitudes in eyes with and without retinal changes. Arden ratios on the electrooculogram were normal in 13 of 14 patients tested. During the follow-up period, which exceeded 5 years, 3 of 9 patients had newly developed or enlarged, patterned changes of the pigment epithelium. These results suggest that pigmentary retinal changes in patients with myotonic dystrophy are slowly progressive.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Electrooculography
  • Electroretinography
  • Female
  • Fluorescein Angiography
  • Follow-Up Studies
  • Fundus Oculi
  • Humans
  • Male
  • Middle Aged
  • Myotonic Dystrophy / complications
  • Myotonic Dystrophy / pathology*
  • Prevalence
  • Retina / pathology*
  • Retinitis Pigmentosa / complications
  • Retinitis Pigmentosa / pathology*
  • Visual Acuity