Leber’s Hereditary Optic Neuropathy
Also known as Leber's Disease, Leber’s optic neuropathy is a painless eye disease typically found in young males. Because it is painless most victims are completely unaware of it until they manifest blurry vision. Four out of five sufferers are boys. Visual encumbrances usually begin in one eye three months prior to the other. If untreated, vision loss continues to legally blindness. In advanced stages cardiac arrhythmias and tremors may also manifest. Leber’s Disease is now understood to be caused by genetic mutations in mitochondrial DNA. Like the baldness gene it is carried by mothers to their sons. It is a curious disease. For instance, is often only transferred to one child in the family. It is so difficult to track because up to 40% of Leber’s suffers have no apparent family history. At-risk family members who are asymptomatic should absolutely consider seeking testing so they can make more informed decisions about having children. Some sufferers, usually female, can develop a multiple sclerosis (MS) like condition. Smoking and alcohol are considered risk factors. Individuals with Leber’s are strongly advised not to drink or smoke. With its ability to positively affect the mitochondria in the cell. Idebenone in capsules or liposomal creams have been shown to be greatly beneficial to Leber’s sufferers, especially in the early stages.
Randomized placebo-controlled trials of idebenone showed idebenone administration to patients with discordant visual acuities at baseline; that all secondary end-points were significantly different between the idebenone and placebo groups. Published on July 25, 2011 in Brain, the Journal of Neurology, the first randomized controlled trial of the mitochondrial disorder for Leber’s optic neuropathy showed evidence that patients with discordant visual acuities were likely to benefit from idebenone treatment and that it was safe and well tolerated. Prenatal testing for at risk parents considering having children is possible via DNA analysis.
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