November 2017
Volume 58, Issue 13
Open Access
Letters to the Editor  |   November 2017
Fuchs' Endothelial and Myotonic Dystrophies: Corneal Dystrophy in Myotonic Patients
Author Affiliations & Notes
  • Maddalena De Bernardo
    Department of Medicine, Surgery and Dentistry, University of Salerno, Salerno, Italy.
  • Nicola Rosa
    Department of Medicine, Surgery and Dentistry, University of Salerno, Salerno, Italy.
Investigative Ophthalmology & Visual Science November 2017, Vol.58, 5838. doi:10.1167/iovs.17-23171
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      Maddalena De Bernardo, Nicola Rosa; Fuchs' Endothelial and Myotonic Dystrophies: Corneal Dystrophy in Myotonic Patients. Invest. Ophthalmol. Vis. Sci. 2017;58(13):5838. doi: 10.1167/iovs.17-23171.

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      © ARVO (1962-2015); The Authors (2016-present)

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We read with great interest the article by Mootha et al.1 concerning a possible correlation between Fuchs' endothelial corneal dystrophy (FECD) and myotonic dystrophy (MD). 
We thank the authors because, among the studies we made trying to understand the reason of low intraocular pressure in patients with MD,26 they cited one study we published in 2010 where we examined the endothelial cells characteristics in these patients.5 
In this study, we found the endothelial cells characteristics to be normal, and we found no patients with FECD. 
Mootha et al. found 46% of their myotonic dystrophy type 1 (DM1) patients affected by FECD and suggested that this difference could be due to the different age of the patients we examined. In fact, our patients presented a mean age of 38 ± 13.3 (SD) years, whereas they screened DM1 subjects over the age of 40 years because FECD is a disease of middle age. 
This could be a correct explanation, but none of our patients older than 40 presented with FECD, and their hypothesis contradicts the paper of Heringer et al.7 that reported two MD patients; one patient was 26 year old. 
It may not be the age, but the severity of the disease or a subset of this disease that was not present in our patients, that could be another explanation for FECD. 
References
Mootha VV, Hansen B, Rong Z, et al. Fuchs' endothelial corneal dystrophy and RNA foci in patients with myotonic dystrophy. Invest Ophthalmol Vis Sci. 2017; 58: 4579–4585.
Rosa N, Lanza M, Borrelli M, et al. Intraocular pressure and corneal biomechanical properties in patients with myotonic dystrophy. Ophthalmology. 2009; 116: 231–234.
Rosa N, Lanza M, Borrelli M, et al. Low intraocular pressure resulting from ciliary body detachment in patients with myotonic dystrophy. Ophthalmology. 2011; 118: 260–264.
Rosa N, Lanza M, De Bernardo M, et al. Intraocular pressure in patients with muscular dystrophies. Ophthalmology. 2013; 120: 1306–1307.
Rosa N, Lanza M, Borrelli M, et al. Corneal thickness and endothelial cell characteristics in patients with myotonic dystrophy. Ophthalmology. 2010; 117: 223–225.
De Bernardo M, Russo V, Rosa N. Ophthalmological findings in myotonic dystrophy. Arq Neuropsiquiatr. 2017; 75: 327.
Heringer JF, Santo RM, Barbosa LJ, et al. Corneal endothelial dystrophy associated with myotonic dystrophy: a report of 2 cases. Cornea. 2017; 36: e24–e25.
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