Neuro-ophthalmological manifestations of POEMS syndrome

The cases is presented of a 38 year-old male with a constitutional syndrome, fever, multiple swollen lymph nodes, and hepatosplenomegaly of 2 months onset. There was also mention of headache, bilateral blurred vision, and myiodesopsias. Best correct visual acuity was 20/50 and 20/200. The anterior segment was unremarkable. The fundus of both eyes showed raised and erased discs, accompanied by serous detachment, greater in the left eye. Complementary studies of both eyes showed a visual field with increased blind spot; fluorescein angiography indicated late disc hyperfluorescence. The electroretinogram showed compromise of cones and rods, and the visual evoked potential detected alteration in the perception and conduction of stimuli. The imaging studies were essentially normal. During his hospital admission he presented with paraparesis, renal failure, endocrinopathy, skin alterations, and osteoblastic lesions with negative biopsies. Monoclonal gammopathy was documented, and the diagnosis of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome was made. The chemotherapy started with dexamethasone/melphalan, with a good response at 3 months.