A 66-year-old man with Diabetes Mellitus developed progressive bilateral blurred vision over a month and was given a diagnosis of proliferative diabetic retinopathy. He received three injections of Aflibercept two weeks apart but his vision continued to deteriorate.
A month later, he presented to the emergency room with gait imbalance, worsening blurred vision and multiple falls. Brain MRI and basic labs were unremarkable. He was discharged to a rehabilitation facility where his vision and cognition deteriorated with development of expressive aphasia and generalized startle myoclonus prompting readmission. EEG showed continuous background slowing. A spinal tap was performed which showed high CSF protein (180 g/dl) but 14-3-3 returned negative. CTA of the head and neck and extensive lab testing including Lyme serology were all unremarkable. Over the next couple of days, he became unresponsive. Repeat MRI brain showed cortical ribbon sign and bilaterally symmetric restricted diffusion in the caudate, putamen and basal ganglia. Repeat EEG showed frequent 1Hz generalized periodic discharges (GPDs). Taken together, patient met CDC’s diagnostic criteria for probable sCJD. He rapidly deteriorated and subsequently died.