Leber hereditary optic neuropathy (LHON): management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Leber hereditary optic neuropathy (LHON): management. There are brain white matter changes: these are also seen in asymptomatic LHON carriers.

Magnetic resonance imaging (MRI)

  • There are brain white matter changes: these are also seen in asymptomatic LHON carriers
  • There is T2 hyperintensity of the posterior optic nerves and the optic chiasm
  • There is no optic nerve enhancement
  • The optic chiasm is enlarged
  • There may be T2 hyperintensities in the substantia nigra: case report
  • It may manifest with longitudinally extensive spinal cord lesions

Triggers for blindness

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Monitoring

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Investigational treatments

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References

  1. Sadun AA, La Morgia C, Carelli V. Leber's hereditary optic neuropathy. Curr Treat Options Neurol 2011; 13:109-117.
  2. Blanc C, Heran F, Habas C, Bejot Y, Sahel J, Vignal-Clermont C. MRI of the optic nerves and chiasm in patients with Leber hereditary optic neuropathy. J Neuroophthalmol 2018; 38:434-437.
  3. Milesi J, Rocca MA, Bianchi-Marzoli S, et al. Patterns of white matter diffusivity abnormalities in Leber's hereditary optic neuropathy: a tract-based spatial statistics study. J Neurol 2012; 259:1801-1807.
  4. Long M, Wang L, Tian Q, Ding H, Qin W, Shi D, Yu C. Brain white matter changes in asymptomatic carriers of Leber's hereditary optic neuropathy. J Neurol 2019; 266:1474-1480.
  5. Echiverri K, Darpel K, Smith C, Sudhakar P. Teaching NeuroImages: Substantia nigra T2 hyperintensities in a man with Leber hereditary optic neuropathy. Neurology 2019; 93:e1830-e1831. 
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