Should 'Retro-ocular Pain, Photophobia and Visual Acuity Loss' Be Recognised as a Distinct Entity? The ROPPVAL Syndrome

Unilateral retro-ocular pain, photophobia and visual disturbance in patients suspected as having acute optic neuritis was described as a distinct clinical entity by Jefferis et al. in 2018. We hereby report a further four patients with the same clinical phenotype and propose the term ROPPVAL syndrome (Retro-Ocular Pain, Photophobia and Visual Acuity Loss). All of them had a previous (mis)diagnosis of optic neuritis. All of the patients had normal ocular and neurological examinations, no relative afferent pupillary defect and no objective structural abnormality was identified. We also discuss possible mechanisms, the role of cycloplegics that we found to be useful in reducing symptoms, and the importance of distinguishing this syndrome from optic neuritis.