The recent observation that patients with Leber’s hereditary optic neuropathy (LHON) can demonstrate dilated microvasculature in both the affected and unaffected subclinical eyes of the same patient revealed the physiological characteristics of bilateral optic disease risk carried in patients suffering from this hereditary mitochondrial condition.

I note that the famed Swiss mathematician Leonhard Euler (1707-1783) suffered from a hereditary ophthalmopathy (Figure) whose characteristics may also correspond to LHON. During his life, Euler suffered from a long-standing familial eye condition. His ancestral surname was sometimes given as Euler-Schölpi, translated as “Euler-squint-eyed” or “Euler-crooked-eyes”. He had sequential visual loss, first at age 31 years (in his right eye) and then at age 59 years (in his left eye). Frederick the Great of Prussia called him “cyclops” and just before the loss of vision in his left eye, it was diagnosed as a cataract. He had 13 children but only 5 survived into infancy, but there is no reported evidence of ophthalmopathy in his sons. Although he is noted to have had an inherited ophthalmopathy, this evidence may suggest a maternal and therefore possibly mitochondrial component.

Together these facts of inherited eye condition with sequential visual loss in adulthood suggest that the most likely differential diagnosis could be LHON. Although the 28-year difference in sequential vision loss between the right and left eye is unusual, there have been reports of long periods between vision loss in each eye associated with this disease.

Additionally, it is reported that Euler eventually died at age 76 years of a brain hemorrhage. This  may have be caused by cerebrovascular disease associated with Euler’s age, but may also represent the increased mortality and increased incidence of stroke (with a relative risk of 2.38 compared with unaffected family members) in patients with LHON.

It is interesting to note that one of the greatest mathematicians of all time suffered from an inherited mitochondrial neuropathy over a century before Theodor Leber (1840–1917) first described this condition in 1871, and reinforces Euler’s brilliance in continually demonstrating mathematical genius despite suffering from neuro-ophthalmological disease.

 

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