Chalky Pallid Edema

A 56-year-old man with unremarkable prior history complained of visual and somatosensory disturbances. Five months prior, he had an episode of being unable to see oncoming traffic. He developed transient visual disturbances lasting minutes in the left hemifield, e.g., the right sides of people's faces looked as if they were melting. His family noticed a tendency to veer toward the left when walking. Optometry evaluation was unrevealing. Four months later he had an episode of sudden difficulty using his left hand, prompting a hospital admission. An MRI brain was performed, and showed multiple areas of restricted diffusion. A workup for stroke etiologies was negative apart from mildly elevated inflammatory markers. Upon discharge, aspirin and atorvastatin were added to his medications. He continued to have episodes of left hand and face numbness about twice weekly and occasional episodes of left-sided visual disturbances. He developed intermittent intense left temple pain occurring 1-2x/week. He presented emergently again with a transient episode of left facial droop, left arm numbness and brief confusion. Neuro-ophthalmic exam revealed: visual acuities of 20/20 OD, 20/25 OS, previously known color blindness, and otherwise excellent afferent and efferent function. Perimetry showed an inferior arcuate defect OS. The fundi appeared normal. OCT showed normal disc and foveal contours but suggested thickened choroid. Fluorescein angiography was notable for delayed A-V and choroidal filling. Repeat brain MRI showed enlargement of the previously noted diffusion-positive areas.; An extensive laboratory evaluation was notable for mildly elevated inflammatory markers and LDH, otherwise negative infectious, autoimmune, paraneoplastic and hypercoagulable studies. LP showed mildly elevated protein and elevated IgG. CT torso showed three distinct lobulated retroperitoneal masses, biopsy of which revealed large B-cell lymphoma. Brain biopsy was performed. This showed minimally hypercellular brain parenchyma with abnormal atypical cells within vessels, establishing a diagnosis of intravascular lymphoma.