Transient aphasia and abnormalities in MRI in a patient with thrombotic thrombocytopenic purpura The acquired thrombotic thrombocytopenic purpura (TTP) is an immune-mediated deficiency of the von Willebrand factor (vWF)-cleaving protease ADAMTS13. This causes unrestrained adhesion of vWF-multimers and activated platelets and leads to microthrombosis with thrombocytopenia, hemolytic anemia and tissue ischemia. We describe the case of a 55-year-old woman, which developed an aphasia and a delirium with abnormalities in MR-imaging as part of TTP. The patient has been in an external hospital due to thrombocytopenia, hematoma of the lower body and hematuria. She developed a TTP and was treated with prednisone. Within some days she got a delirium and an aphasia. So the patient was transferred to our hospital in order to implement plasmapheresis. We saw an awake patient with aphasia but no paresis or sensory disturbance. In native CT-imaging and CT angiography there was no hint for cerebral infarction or vessel occlusion. Parameters of hemolysis and creatinine were highly elevated. So we started a plasmapheresis. The MR-imaging the same day revealed subacute ischemias in both thalami and in the left hemisphere with DWI-lowering and flair hyperintensities. The ADAMTS13-activity was lower than 0,8% what proved the diagnosis of TTP. Due to plasmapheresis the patient improved in neurological status and hemolysis parameters and renal function recovered. The aphasia was nearly gone when she was transferred to the clinic of internal medicine for continuitation of plasmapheresis. Further treatment was implemented with prednisone and Rituximab. Even the abnormalities in MRI disappeared completely. The patient has been discharged from hospital after about 3 weeks.
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