TH-207. A particular case of Sturge weber syndrome revealed in adulthood, implications of the COVID-19 era

ABSTRACT

Sturge weber syndrome (SWS) is a rare angiomatosis described in the pediatric population. There are only few cases with a late revelation in adulthood. We report a particular adult case of SWS revealed after a second dose of mRNA-COVID-19 vaccination. A 61-year-old man with cardiovascular factors presented with a set of escalating neurological symptoms (blurred vision, hemiparesis, headaches, CBH, convulsions, movement disorders) a month after he received his second dose of the Pfizer-BioNTech COVID-19 vaccine. Susceptibility weighted imaging on brain MRI showed enlarged transmedullary veins in the right parieto-occipital lobes as well as contrast enhancement of the same localization after gadolinium injection compatible with the diagnosis of cerebral angiomatosis. Lumbar puncture revealed a slight elevation of protein. Multiple EEGs and a video-monitoring EEG showed alternate, predominantly right hemisphere seizures not always accompanied by clinical manifestations. The evolution was marked by refractory seizures to anti-epileptic drugs and movement disorders only stabilized after 10 g of methylprednisolone. A complete panel of investigations was done to look for autoimmune or infectious encephalitis, metabolic encephalopathy, and systemic malignancy but all were negative. Clinical presentation of SWS includes a wide range of neurological symptoms (stroke-like episodes, migraine, blurred vision, seizures, etc.). Our patient had many of these symptoms and is classified as a type III SWS on the Roach Scale. However, the presence of movement disorders in this condition is not reported. The worsening of the patient under anti-epileptic medication was another red flag to the diagnosis of isolated SWS. Since the patient's recovery was only obtained after immunosuppressive therapy and no biological evidence of any other etiology of encephalopathy was found, the hypothesis of an autoimmune encephalitis on a fragile brain triggered by COVID-19 vaccination was the most probable explanation to this presentation. Throughout this case, we wanted to report another case of late-onset SWS meanwhile highlight the importance of further exploration of the possible connection between COVID-19 vaccination and autoimmune encephalitis. Copyright © 2022