PEDIATRICS Vol. 91 No. 5 May 1993, pp. 988-989
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Leber's Hereditary Optic Neuropathy as a Cause of Severe Visual Loss in Childhood

C. M. MOORMAN FCOPHTH1, J. S. ELSTON FCOPHTH1, and P. MATTHEWS MD, DPHIL2

1 Oxford Eye Hospital, Oxford, England
2 Genetics Laboratory and University Dept of Neurology, Oxford University, Oxford, England

Leber's hereditary optic neuropathy (LHON) is a rare, maternally transmitted disease that most commonly causes acute or subacute visual loss in young men, typically between the ages of 17 and 24 years (although perhaps 14% of affected individuals are women), which may be associated with systemic disorders, eg, cardiac dysrhythmias and neurologic problems.1 Onset is usually asymmetric, but intervals between involvement of the two eyes are usually less than a few months. A definitive diagnosis rested on a family history, age of onset, and the characteristic circumpapillary microangiopathy of the optic disc in the acute phase. However, recent demonstration of point mutations of mitochondrial DNA in affected individuals means that confirmation of the diagnosis can now be obtained in atypical or sporadic cases.2

Submitted on October 12, 1992
Accepted on December 4, 1992




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