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Eye Findings

Alfredo A. Sadun, MD, PhD; James F. Martone, MD; Rafael Muci-Mendoza, MD; Lillian Reyes; Lindreth DuBois, MEd; Juan Carlos Silva, MD; Gustavo Roman, MD; Benjamin Caballero, MD, PhD

Arch Ophthalmol. 1994;112(5):691-699.

Abstract
Objective
To characterize and establish a clinical definition of the optic neuropathy that appeared in epidemic form in Cuba in 1992 and 1993.

Methods
At the invitation of the Cuban Ministry of Health, Havana, members of ORBIS International and the Pan American Health Organization, assembled teams that traveled to Cuba in May 1993. We were initially briefed by Cuban national experts in the areas of virology, nutrition, toxicology, ophthalmology, neurology, and public health. We then examined 20 patients on our own. Thirteen of these patients underwent a comprehensive neuroophthalmologic examination, including neurologic examination, ophthalmologic examination, visual fields, optic nerve function studies, contrast sensitivity studies, and funduscopy. We returned 4 months later to perform an additional 12 comprehensive neuro-ophthalmologic and follow-up examinations.

Results
Only seven of the 13 patients who were alleged to have the optic form of the epidemic and who were rigorously and systematically examined on the first visit demonstrated a bilateral optic neuropathy. These seven patients had several features that included decreased visual acuity, poor color vision, central scotomas, decreased contrast sensitivity, saccadic eye movements, and most prominent and distinctive of all, nerve fiber layer wedge defects of the papillomacular bundle. Our clinical definition was then implemented by the Cuban ophthalmologists and epidemiologists. On returning 4 months later, we found that all newly presented patients were correctly diagnosed to have the epidemic disease. With the new case definition and the application of a few simple psychophysical tests, the false-positive rate of diagnosis became much lower. After vitamin therapy, we reexamined the patients seen on our initial visit, and all showed marked improvement.

Conclusions
The Cuban epidemic was characterized by an optic neuropathy with features that were similar to those of tobacco/alcohol amblyopia and Leber's optic atrophy. Recent political, economic, and social changes in Cuba may have contributed to the nutritional and/or toxic compromise of mitochondrial function of an acquired nature, which led to selective retinal ganglion cell damage. We have termed this condition Cuban epidemic optic neuropathy.

Author Affiliations
From ORBIS International, New York, NY (Drs Sadun and Martone and Ms Reyes), the Pan American Health Organization, Washington, DC (Drs Muci-Mendoza, Silva, Roman, and Caballero), the Doheny Eye Institute, the University of Southern California School of Medicine, Los Angeles (Dr Sadun and Ms Reyes), Emory University, Atlanta, Ga (Ms DuBois), Universidad Central de Venezuela, Caracas (Dr Muci-Mendoza), and The Johns Hopkins School of Hygiene and Public Health, Baltimore, Md (Dr Caballero).

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