A case of Churg–Strauss syndrome and central retinal artery occlusion with good visual recovery
A 58-year-old man had been diagnosed with CSS from bronchial asthma, eosinophilia, polyneuropathy, and infiltration in lungs 2 years before. Bilateral foot drop and hypereosinophilia developed 2 weeks before the present condition and he was treated with oral prednisolone (15 mg/day). On referral to the Department of Neurology of our hospital, he had a diagnosis of peroneal nerve paralysis by CSS and steroid pulse therapy (methylprednisolone at 1 g/day) was started. The following day, he experienced acute painless loss of vision in his right eye and was immediately admitted to the Department of Ophthalmology.\nOn initial examination, the best corrected visual acuity was hand motion on the right side and 20/20 on the left. Slit-lamp examination revealed bilateral early cataracts. There was marked pallor of the retina with a cherry-red spot in the fovea on fundoscopy of the right eye []. Fluorescein angiograms showed delayed arterial filling in the right eye []. Eyeball massage and anterior chamber paracentesis were performed as initial treatment, but retinal perfusion did not recover. Laboratory tests showed a white blood cell count of 15,250/mm3, with hypereosinophilia (24%). C-reactive protein (reference range is 0.3 mg/dl or less) increased to 6.4 mg/dl. Perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) and cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) were negative. Because it was thought that the patient's CRAO had been caused by an inflammatory process, steroid pulse therapy (methylprednisolone at 1 g daily for 3 days) was continued and was followed by combined treatment with oral prednisolone (30 mg/day) and low-dose cyclophosphamide (150 mg/day) together for management of intractable CSS. There was rapid resolution of foot drop and normalization of hypereosinophilia. His visual acuity improved more slowly and recovered to 20/30 one month after CRAO. Result of fluorescein angiography performed at that time showed resolution of the retinal ischemia of the right eye [].\nOral prednisolone was tapered slowly to 10 mg/day over 6 months. No recurrence has occurred over 18 months, and the most recent visual acuity was 20/30.