Acute posterior multifocal placoid pigment epitheliopathy associated with CN III palsy

PURPOSE: To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cranial nerve (CN) III palsy. OBSERVATIONS: A 20-year-old woman developed bilateral anterior uveitis, which resolved with topical steroids. Three weeks later she exhibited posterior pole lesions in both eyes, corresponding with a diagnosis of APMPPE, as confirmed by multimodal imaging. Two days later the patient presented with right CN III palsy. The patient was started on oral prednisone, which was gradually tapered off. Signs and symptoms improved rapidly, with complete resolution within two months. CONCLUSION AND IMPORTANCE: Though rare, APMPPE may present with neurological involvement, as in this previously unreported association with CN III palsy. Unlike uncomplicated APMPPE cases, in patients with neurological manifestations systemic therapy is advocated.