Recommended Reading – Toxic medications in Leber’s hereditary optic neuropathy

Recommended Reading – Toxic medications in Leber’s hereditary optic neuropathy

Toxic medications in Leber’s hereditary optic neuropathy.
Kogachi K, Ter-Zakarian A, Asanad S, Sadun A, Karanjia R
Mitochondrion. 2018 Aug 4. pii: S1567-7249(18)30036-9. doi: 10.1016/j.mito.2018.07.007. [Epub ahead of print]

Leber’s hereditary optic neuropathy (LHON) is a maternally inherited mitochondrial disorder characterized by acute bilateral vision loss. The pathophysiology involves reactive oxygen species (ROS), which can be affected by medications. This article reviews the evidence for medications with demonstrated and theoretical effects on mitochondrial function, specifically in relation to increased ROS production. The data reviewed provides guidance when selecting medications for individuals with LHON mutations (carriers) and are susceptible to conversion to affected. However, as with all medications, the proven benefits of these therapies must be weighed against, in some cases, purely theoretical risks for this unique patient population.


… In this article, we review medications that are proposed to increase ROS. Given the association between ROS and conversion, understanding the effects of these agents on mitochondria may assist clinicians to understand the implications of these classes of medications for patients with LHON. Medications on this list can be used in patients with LHON, especially when no alternative exists. As with all therapies, a balance must be struck between the therapeutic benefits and potential harm, which may warrant special consideration for patients with LHON.

Full Text: