Atypical posterior reversible encephalopathy syndrome in a case of thrombotic thrombocytopenic purpura with Sjogren's syndrome

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder characterized by thrombotic microangiopathy. Neurologic symptoms are frequently seen in its presentation and the most common finding on neuroimaging of TTP is posterior reversible encephalopathy syndrome (PRES). Acute strokes, hemorrhages and atypical PRES are uncommonly seen. Our case reports the clinical and imaging details of a young male patient with TTP and Sjogren's syndrome, who made a complete recovery after aggressive plasmapheresis and immunosuppressive therapy with resolution of the imaging findings of PRES on follow up brain MR imaging. We briefly review the literature for the spectrum of imaging findings that can be seen on brain MRI with TTP.

Sjögren's syndrome presenting as thrombotic thrombocytopenic purpura in a male patient with previous Kikuchi-Fujimoto disease

“…A review identified 18 cases of concomitant SS and TTP (16 in primary SS) (2). Four cases were reported since (3)(4)(5)(6). In these 20 primary SS patients, most were female (80%) and one-third (n=7/20) had SS diagnosed prior to TTP.…”