A Rare Case of Unilateral Progressive Vision Loss and Pachymeningitis.

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.

Longitudinally extensive optic neuritis in neuromyelitis optica spectrum disorder.

BACKGROUND: Neuomyelitis optica, sarcoid, and multiple sclerosis can all cause optic neuritis. Further means of distinguishing the causes of optic neuritis among these etiologies would be valuable for the clinician. METHODS: This is a retrospective, cohort study from a single university based hospital and neuro-ophthalmology clinic. Blinded interpretation of orbit MRIs was performed on patients with acute optic neuritis from multiple sclerosis (n=25), sarcoid (n=5) and neuromyelitis optica spectrum disorder (n=6). RESULTS: A length of >40 mm anterior visual pathway enhancement distinguished neuromyelitis optica spectrum disorder from multiple sclerosis (p=0.0376). No statistically significant differences were found for presence of pain or papillitis, however there was a trend for bilateral involvement and chiasmal involvement in neuromyelitis optica spectrum disorder compared to multiple sclerosis. CONCLUSIONS: In acute optic neuritis, enhancing anterior visual pathway lesion length >40 mm helps differentiate neuromyelitis optica spectrum disorder from multiple sclerosis. This degree of involvement can be considered longitudinally extensive optic neuritis. Further characterization is necessary as this degree of enhancement occurs in other clinical syndromes besides neuromyelitis optica.

Corneal confocal microscopy is efficient, well-tolerated, and reproducible.

In order to develop an efficient, reproducible, and well-tolerated protocol for assessing corneal innervation, 11 normal subjects underwent corneal confocal microscopy (CCM) using a Heidelberg Retinal Tomography III microscope. Five standardized locations were sampled in the left eye and one centrally in the right. The protocol was repeated 1-4 weeks later. A blinded technician measured nerve fiber length (NFL) and tortuosity coefficient (TC). The relationship between image location and NFL and TC was assessed using one-way analysis of variance, and reproducibility determined using relative intertrial variability and intraclass correlation coefficients. NFL reproducibility was maximized by averaging four or more images from the left eye, or one central image from both eyes. TC was less reproducible. CCM is a rapid, well-tolerated, and reproducible method for assessing corneal innervation.