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Retinal changes in myotonic dystrophy. Clinical and follow-up evaluation
Y Kimizuka1, M Kiyosawa, M Tamai
1Department of Ophthalmology, Tohoku University School of Medicine, Japan.
Retina (Philadelphia, Pa.)
|January 1, 1993
Summary
Retinal changes are common in myotonic dystrophy patients, affecting the macula and periphery. These pigmentary changes appear to progress slowly over time.
Area of Science:
- Ophthalmology
- Genetics
- Neurology
Background:
- Myotonic dystrophy is a multisystem disorder with ocular manifestations.
- Retinal changes, including pigmentary abnormalities, are known complications.
Purpose of the Study:
- To determine the prevalence and progression of retinal changes in a large cohort of myotonic dystrophy patients.
- To characterize the specific types and locations of these retinal alterations.
Main Methods:
- Ophthalmic examination of 49 patients with myotonic dystrophy.
- Assessment of macular, midperipheral, and peripheral retinal changes.
- Electroretinography (ERG) and electrooculography (EOG) were performed.
- A follow-up period exceeding 5 years was utilized to evaluate progression.
Main Results:
- Prevalence: 26.6% butterfly-shaped macular changes, 24.5% reticular midperipheral changes, 43.9% peripheral atrophic changes.
- 18 eyes (10 patients) exhibited two distinct types of retinal changes.
- Visual acuity was generally preserved; ERG showed diminished a and b waves.
- Over 5 years, 3 of 9 patients showed new or enlarged pigmentary changes.
Conclusions:
- Pigmentary retinal changes are frequent in myotonic dystrophy.
- These retinal alterations demonstrate slow progressive characteristics.
- Ocular findings in myotonic dystrophy warrant long-term monitoring.