Coming to a Rapid Conclusion - Video

Head CT showed diffuse effacement of the sulci with diffuse cytotoxic edema and cerebral edema in a right middle, left middle, and left posterior cerebral artery distribution. He was started on mannitol and hypertonic saline. MRI of the brain showed large areas of ischemia in the bioccipital, biparietal, and right frontal regions with associated cytotoxic edema. MR Angiogram showed a left M1 occlusion. Lumbar puncture could not be performed in the setting of active herniation. His family declined decompressive bifrontal craniectomies due to his poor prognosis. Over the next 24 hours, he progressed to brain death despite aggressive treatment with mannitol and hypertonic saline. This was 42 days after the onset of visual changes, and 24 days after his initial visit to a retina specialist. On autopsy, ocular pathology showed granulomatous choroiditis with multinucleated giant cells confined to the posterior pole, with some foci of overlying subretinal fluid (correlating with the active-appearing subretinal infiltrates noted on funduscopic examination, choroidal thickening noted on OCT, and hypofluorescent areas on fluorescein angiogram), with adjacent areas of RPE atrophy (correlating with the areas of RPE atrophy noted on funduscopic examination, OCT, and fluorescein angiogram). HSV-1 and HSV-2 immunostains were negative. In the brain and spinal cord, blood vessels of all sizes, including left MCA tributaries, showed patchy chronic inflammation varying from lymphocytic to lymphohistiocytic to granuloma formation; granulomatous vasculitis was best appreciated in the spinal cord. Some inflamed vessels also displayed focal associated angionecrosis and partially organized luminal thrombi. Most foci of vasculitis were observed in leptomeninges with limited involvement of intraparenchymal vessels. Also noted were multiple ischemic infarcts, evidence of cerebral edema, and Duret hemorrhage.