Leber hereditary optic neuropathy (LHON): management

Evidence-based neurology checklist on leber hereditary optic neuropathy (lhon): management: 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…

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

Monitoring

Investigational treatments

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. 
  6. And 13 more. Subscribe to see the full list

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