Undifferentiated uveomeningoencephalitic syndrome requiring systemic immunosuppression: A case-report

Poster Details

First Author: D.Sousa PORTUGAL

Co Author(s):    P. Magro   I. Leal                          

Abstract Details


To report a case of a 26-year-old woman who was referred due to severe headache, nausea and bilateral anterior uveitis.


Ophthalmology Dept., Hospital de Santa Maria, Lisboa, Portugal.


Retrospective and descriptive case-report based on data from clinical records and analysis of ancillary studies.


A 26-year-old black woman presenting with severe headache and nausea was admitted. Best-corrected visual acuity (BCVA) was 20/20 OU. Slit-lamp examination revealed bilateral cilio-conjuntival hyperemia, unremarkable cornea, anterior chamber cell 3+ and flare 2+ and an anterior vitritis 1+. Pupillary function, intraocular pressure and fundoscopy were normal. All blood/liquor serologic tests for infectious pathologies were negative. The patient was kept on topical therapy for anterior uveitis, but systemic and ophthalmologic symptoms worsened significantly in following days: BCVA 20/60-OD and 20/32-OS, granulomatous keratic precipitates were distinguishable, anterior cell/flare and vitritis. Vogt-Koyanaga-Harada (VKH) disease was suspected. Ocular coherence tomography and ultrasound revealed a slightly increased choroid thickness and were otherwise normal. Fluorescein angiography was normal but electroretinogram revealed bilateral cone/rod dysfunction. Bilateral sensorineural hearing loss was diagnosed on ENT consultation, head MRI revealed a puntiform inflammatory lesion in the pons, dermatologic consultation excluded skin lesions and HLA-DR01*04 was negative. Causes of granulomatous uveitis, such as sarcoidosis and infectious causes were all ruled out. High-dose systemic corticosteroid treatment along with oral methotrexate (20 mg/week) and cyclosporine A (100 mg bid) was given and symptoms improved steadily, with a final BCVA of 20/20 OU and complete quiescence of the intraocular inflammation.


After an extensive work-up for causes of granulomatous uveitis in this patient, no clear diagnostic entity was found and a diagnosis of undifferentiated uveomeningoencephalic syndrome was made. This led us to the decision of systemic immunosuppression with significant clinical improvement. This case-report emphasizes the importance of promptly deciding and mastering conventional immunosuppression in ocular inflammation settings, even when a final diagnostic entity is not found.

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