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Retin Cases Brief Rep. 2022 May 19. doi: 10.1097/ICB.0000000000001315. Online ahead of print.

ABSTRACT

ABSTRACT: Purpose: To report a case of nonparaneoplastic autoimmune retinopathy in a patient with diagnosis of Lambert Eaton Myasthenic Syndrome.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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.

PMID:36007183 | DOI:10.1097/ICB.0000000000001315


Source: https://pubmed.ncbi.nlm.nih.gov/3600718 ... 7&v=2.17.7