A MIDDLE-AGED PATIENT WITH BILATERAL VISION LOSS AND NYCTALOPIA.

Abstract

Purpose: To report a case of nonparaneoplastic autoimmune retinopathy in a patient with diagnosis of Lambert Eaton Myasthenic Syndrome. Case report. Main outcome measures included findings on retinal examination and analysis of fundus autofluorescence, spectral domain optical coherence tomography, and full field electroretinogram. Vitamin A levels and results of antiretinal antibody testing and paraneoplastic work up are also presented. A 47-year-old male presented with a 1-year history of bilateral vision loss and nyctalopia. Past medical history was significant for Lambert-Eaton myasthenic syndrome (LEMS), confirmed by positive voltage-gated calcium channel (VGCC) antibodies, and thymectomy reported as thymic follicular hyperplasia. OCT showed bilateral diffuse outer retinal atrophy and ellipsoid zone loss. Fundus autofluorescence displayed a bull's pattern of hyperautofluorescence around each fovea. Full field ERG showed an extinguished rod response and a severely depressed cone response in each eye. We describe a case of nonparaneoplastic autoimmune retinopathy in a patient with Lambert Eaton Myasthenic Syndrome. Multimodal retinal imaging and ERG confirmed the presence of autoimmune retinopathy with severe rod-cone degeneration. The association of this myasthenic syndrome with AIR is novel.