First Author: K.Theodoraki UK
Co Author(s): S. Antonakis A. Goncalo
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To present a case of a patient with paraneoplastic optic neuropathy confirmed with anti-Enolase antibodies
The patient presented through the eye casualty in Maidstone Hospital, Kent.
Retrospective review of case notes
A 69 year old lady was seen in the eye casualty with reduced vision of the left eye over weeks. She had a background of breast cancer treated manages surgically 6 years ago. She was recently diagnosed with metastatic disease in the kidneys for which she received chemotherapy; later discontinued due to poor tolerance. On examination her vision was OD 6/9 and OS 6/60.She had absent colour vision in the left eye and normal in the right.Humphrey 24-2 visual field revealed complete scotoma in the left eye with mean deviation of -29Db. In the right eye she had a superior scotoma with MD -4.96Db. Clinical examination showed normal IOP with open angles and bilateral RPE atrophy(previous central serous retinopathy).Optic nerve appeared healthy bilaterally. Her fluorescence angiogram was normal. Antiretinal antibodies were positive for enolase.The patient underwent electrodiagnostic testing. Flash VEPs were undetectable from the left eye while photopic flicker ERGs were of slightly lower amplitude. Testing was interrupted as the patient developed seizures; she was found to have metastatic deposits to the brain with no orbital involvement. The diagnosis of paraneoplastic optic neuropathy was made. The patient was managed with high dose of oral steroids and retained some of her vision until deceased a few months later.
Paraneoplastic optic neuropathy is a rare paraneoplastic syndrome; auto-antibodies are directed against retinal antigens, leading to cell apoptosis and retinal degeneration. Symptoms can be subtle with visual loss occurring over months. There are no set diagnostic criteria. In a patient with visual symptoms and history of cancer the diagnosis is aided by several investigations. Those include abnormal electroretinograms, visual field scotomas and positive serum retinal autoantibodies. Management with immunosuppression is the mainstay of treatment , but most of the evidence collected is anecdotal.