Purpose: To present the first reported case of paracentral acute middle maculopathy in association with Susac syndrome.
Method: Case report.
Results: A young female patient presented with unilateral sudden loss of vision of her right eye to count fingers. There were numerous cotton wool spots in a multifocal pattern concentrated around the right macula and optic disk. Clinical examination and spectral domain optical coherence tomography imaging were consistent with paracentral acute middle maculopathy, and fluorescein angiography showed features of retinal arteriole vasculitis. Magnetic resonance imaging demonstrated multiple white matter lesions in a pericallosal distribution. High-dose corticosteroid therapy was commenced, and visual acuity gradually improved to 6/6 corrected. The patient reported hearing impairment and was diagnosed with Susac syndrome for which she has responded extremely well to long-term immunosuppressive therapy.
Conclusion: Paracentral acute middle maculopathy can occur in association with Susac syndrome. Susac syndrome may present only with multifocal retinal ischemia, and such patients with paracentral acute middle maculopathy should be checked for characteristic magnetic resonance imaging lesions and hearing loss.