Look in the Back (Slides)

A 59-year old man with Graves disease developed increasing proptosis and diplopia. Attributing these abnormalities to worsening of the Graves disease, an ophthalmologist treated him with high-dose oral and intravenous corticosteroids. When improvement did not occur, he underwent orbital x-irradiation (unknown dose). He developed new bilateral hearing loss, bilateral lower extremity weakness, and urinary retention eliciting catheter placement. Visual acuity declined, so he was referred to our institution for urgent orbital decompression. When we first encountered him, he was confused and drowsy. Best-corrected visual acuity was 20/80 in the right eye and 20/200 in the left eye. There was no afferent pupillary defect. External examination revealed marked proptosis. Extraocular motility was reduced in all directions. There was marked superficial punctate keratopathy bilaterally. Ophthalmoscopy through dilated pupils revealed multifocal white/yellow subretinal lesions without vitreous cells in both eyes. He had bilateral sensorineural hearing loss and bilateral lower extremity weakness with diminished lower extremity deep tendon reflexes. Orbital CT revealed bilaterally enlarged extraocular muscles with fullness at the orbital apex but no clear compression of the optic nerves. Brain MRI revealed diffuse intracranial leptomeningeal enhancement and enhancement of both auditory canals. Additionally, there was; restricted diffusion in the left fornix and right putamen. Spine MRI revealed enhancement of the cauda equina roots. Lumbar puncture showed a normal opening pressure with a white blood cell count of 578 (78% lymphocytes), a protein of 348 mg/dL, and negative cytology. The leading diagnosis was non-Hodgkin lymphoma (NHL). The putaminal and fornix abnormalities, believed to be; consistent with ischemic stroke, led to more detailed review of the brain MRI, which disclosed vessel wall enhancement of the right internal carotid artery. A procedure was performed