A Rare Case of Unilateral Progressive Vision Loss and Pachymeningitis

ABSTRACT

We describe a 32-year-old man with presumed Vogt-Koyanagi Harada (VKH) syndrome, whose presenting symptoms were headache and progressive loss of vision in the right eye. Neuro-ophthalmic examination showed anterior and posterior uveitis, and retinal detachment in the right eye. Ocular coherence tomography (OCT) showed extensive submacular fluid in the right eye, while the fundus fluorescein angiogram (FFA) confirmed perifoveal retinal pigment epithelium (RPE) disruption and multifocal fluorescein leakage in the right eye. The brain MRI showed a small crescent of dependent fluid layering in the right posterior globe adjacent to the right optic nerve head, and pachymeningeal enhancement of the skull base dura along the clivus.This case demonstrates the utility of brain MRI and OCT findings in the early diagnosis of VKH syndrome, in the absence of prominent clinical signs of meningitis. Aggressive treatment is critical to preserve vision and prevent development of other systemic complications of the disease.

KEYWORDS:

• Pachymeningitis
• progressive visual loss
• Vogt-Koyanagi-Harada (VKH) disease

Declaration of interest

All authors examined and treated the patient and contributed to writing the manuscript. Jorge C. Kattah, MD, has been a consultant for Biogen, Bayer, and Teva. There are no specific disclosures in relation to this report.

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.