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To elucidate the cause(s)of acute or subacute bilateral simultaneous optic neuropathy(BSON)in adult life,a follow up study of 23 patients was performed with clinical assessment,brain MRI,HLA typing,and mitochondrial DNA analysis.The results of CSF electrophoresis were available from previous investigations in 11 patients.At follow up,five(22%)had developed clinically definite multiple sclerosis,four(17%)had mitochondrial DNA point mutations indicating a diagnosis of Leber's hereditary optic neuropathy(LHON).It is concluded that:(a)about 20%of cases of BSON without affected relatives are due to LHON;(b)multiple sclerosis develops after BSON in at least 20%of cases,but the long term conversion rate is likely to be considerably less than the rate of over 70%seen after an episode of acute unilateral optic neurosis in adult life. |
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