April 05, 2010
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Slow-developing thickening of nerve fiber layer in Leber’s optic neuropathy could represent potential for rescue therapy

Ophthalmology. 2010;117(3):623-627.

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The late involvement of the superior and nasal quadrants in eyes with Leber’s hereditary optic neuropathy may represent “a therapeutic window of opportunity,” according to a study.

In six eyes of four patients known to carry the 11778/ND4 homoplasmic mutation on mitochondrial DNA that confers Leber’s hereditary optic neuropathy, optical coherence tomography showed an initial increase in retinal nerve fiber layer thickness in the inferior and temporal quadrants from when patients were presymptomatic to the time of disease onset, defined as vision loss, a mean 8.3 months later.

Thickening of the inferior region persisted until about 3 months after onset of disease. At 3 months after the time of disease onset, retinal nerve fiber layer thickness was more pronounced in the superior and nasal quadrants, according to the study.

The slow onset of thickening in the inferior region represents somewhat of a disconnect, the study authors said, in that what is perceived clinically as a sudden catastrophic event is actually a slow structural change.

“We hope that this longer interval of time will represent a wider therapeutic window of opportunity,” they said.