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Polycythemia Vera, a clonal stem cell disorder, produces neurologic problems in 50-80% of patients. Here, we present the case of a 63 y.o. woman with recurrent episodes of global aphasia and encephalopathy leading to the diagnosis of Polycythemia Vera along with unique MRI findings.

The patient presented with global aphasia, confusion, and mild RUE weakness. She had three similar episodes of inability to speak over the past few months, the longest lasting three days with spontaneous resolution. Brain MRI revealed T2 hyper-intensity of the bilateral caudate heads along with diffuse areas of cortical restricted diffusion (not corresponding to an arterial territory) concerning for encephalitis, metabolic disturbances, or CJD. Her relapsing and remitting course, combined with negative CSF studies (14-3-3<0.2), excluded CJD. Additional workup including CSF studies showed Protein (35), Glucose (50), only three cells along, and negative autoimmune and paraneoplastic antibody panels. Continuous EEG monitoring showed Left>Right hemispheric slowing in theta/alpha frequencies. Her hemoglobin was 21 g/dl, and Hematocrit 61%. Further workup revealed Erythropoietin (1.8) and JAK 2 testing (pending), LDH 259. She received therapeutic phlebotomy and experienced significant improvement over five days with complete resolution of encephalopathy and at discharge, only minor word-finding difficulty.

Polycythemia Vera can cause endothelial damage, sluggish blood flow, and impaired glucose and oxygen metabolism which could produce the cortical and deep grey matter signal changes on her MRI as well as her reversible clinical manifestations.