Extensive brain stem lesions in thrombotic thrombocytopenic purpura: repeat magnetic resonance findings

The authors report on an unusual case of extensive brain stem lesions as a manifestation of thrombotic thrombocytopenic purpura (TTP). A 28-year-old woman developed rapidly progressive neurologic deficits 5 days after a cesarean delivery. Her condition had been normal after delivery. Initial magnetic resonance imaging (MRI) revealed extensive T2 hyperintense lesions involving the entire brain stem; only part of the pons showed hyperintense abnormalities in a concomitantly taken diffusion-weighted image. The hematologic evaluations and her clinical course revealed the diagnosis of TTP, so plasma exchange and methyl-prednisolone therapy were initiated. After 10 days of treatment, she developed neurologic improvement. A follow-up MRI on the 75th day revealed dramatically reduced brain stem lesions with only residual punctate lesions in the pons. Her remaining neurologic deficits were dysarthria, limb ataxia, and left hemiparesis. As demonstrated in this study, extensive brain stem involvement should be added as a possible neuroimaging feature of TTP.