AI Article Synopsis

  • Leber hereditary optic neuropathy (LHON) is a rare genetic disorder mainly affecting young men, leading to vision loss due to optic nerve degeneration, often misdiagnosed as optic neuritis.
  • A case study of an 18-year-old Malay male with a specific LHON mutation showed unexpected vision recovery over three years, highlighting the importance of genetic testing for accurate diagnosis and tracking potential outcomes.
  • The findings emphasize the significance of early diagnosis, genetic counseling, and supportive care in managing LHON, which can improve patients' quality of life despite the unpredictability of the condition.

Article Abstract

Leber hereditary optic neuropathy (LHON) is a rare maternally inherited mitochondrial disorder that predominantly affects young men, leading to optic nerve degeneration and subsequent vision loss. The rarity of LHON and its clinical similarity to optic neuritis complicates diagnosis, necessitating genetic testing to confirm specific point mutations and predict visual outcomes. We report a rare case of an 18-year-old Malay male with m.14484T>C/MT-ND6 mutation of LHON, who demonstrated remarkable spontaneous visual recovery over a three-year follow-up period. This report highlights the pivotal role of genetic testing in diagnosing LHON, explores the variability in visual outcomes associated with different mutations, and underscores the potential for spontaneous recovery in specific mutation variants. Early diagnosis, genetic counseling, and supportive management are critical for optimizing outcomes and improving quality of life.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11550097PMC
http://dx.doi.org/10.7759/cureus.71210DOI Listing

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