Atypical visual loss in giant cell arteritis

Three patients with atypical ocular involvement due to histologically verified giant cell arteritis are reported. Prior to diagnosis, the first patient had periods of amaurosis fugax. He presented with normal vision. In spite of high-dose systemic corticosteroid therapy, he became blind in the terminal stage of his disease due to bilateral occipital cortex infarctions, verified by CT-scan. Autopsy revealed involvement of several intracranial arteries. In case No. 2 there was severe unilateral visual loss and cotton-wool exudates in both eyes. Central vision recovered after corticosteroid therapy; in our experience this is unusual. In case No. 3 irreversible unilateral visual loss was typical for GCA, but the association with polyneuropathy unique. Neurological remission coincided with systemic corticosteroid therapy.