生物学と医学

概要

Optic Neurodegeneration: Time to Act

Nuri Gueven

Optic neuropathies are frequently associated with mitochondrial dysfunction and the associated vision loss has a severe impact on the patient’s quality of life. Our understanding of disease progression of one of the most frequent mitochondrial disorders, Leber’s hereditary optic neuropathy (LHON), together with results of recent clinical trials might provide us with new insights that are relevant not only to the progression of the disease but more importantly also for therapeutic intervention. One of the crucial hallmarks of LHON is the occasional recovery of vision in some patients. This rare and spontaneous process highlights that blindness in LHON patients is not irreversible per se and suggests that this process could potentially be induced by pharmacological intervention. Strikingly, spontaneous recovery of vision has been reported in some patients several years after disease onset, which indicates the presence of an extended time window where recovery is still possible, before over time the terminal loss of retinal neurons renders visual recovery impossible. Several recent encouraging trials in LHON and related disorders support this view and extend this model to other optic neuropathies that are not associated with spontaneous recovery. This concept provides hope not only to mitochondrial optic neuropathy patients, but also to patients that suffer from one of the major ocular disorders such as glaucoma.