Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis.

Abstract

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.

• Authors: Henry W Zhou, Ann Q Tran, Andrea A Tooley, Jeremy T Miyauchi, Michael Kazim
• Journal: Ophthalmic plastic and reconstructive surgery (Ophthalmic Plast Reconstr Surg) 2021 May-Jun 01 Vol. 37 Issue 3S Pg. S160-S162 ISSN: 1537-2677 [Electronic] United States
• PMID: 32991499 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Topics

• Adult
• Cogan Syndrome (diagnosis, drug therapy)
• Dacryocystitis (diagnosis, drug therapy)
• Eye
• Humans
• Male
• Oculomotor Muscles
• Orbital Myositis (diagnosis, drug therapy)