Neuropsychiatric symptoms comprise one of the five classic symptoms of autoimmune
thrombotic thrombocytopenic purpura (aTTP). Although aTTP is typically transient, it is sometimes complicated by
cerebral infarction with residual disability. This report presents the case of an 87-year-old man previously admitted to a different hospital with
fever and transient consciousness loss. After receiving
platelet transfusion with diagnosis of
Evans syndrome, he was transferred to our hospital with worsening consciousness disturbance. He was subsequently diagnosed with aTTP with a PLASMIC score of 6 points, ADAMTS13 activity of less than 0.5%, and its inhibitor of 7.4 BU/ml. Platelet count and consciousness were rapidly improved with
plasmapheresis and
steroids, but
motor aphasia emerged. MRI showed multiple
cerebral infarctions, including a large
infarction in the left frontal lobe. Thus,
unfractionated heparin was administered. When his platelet count dropped once again on the 20th day,
rituximab was added. The treatment eventually proved to be successful, and his aTTP remained in remission one year after the onset. Treatment for
cerebral infarctions was switched to DOAC, and rehabilitation was continued. However, his
ADL has not yet recovered. Advances in aTTP treatment have cured many similar cases. Thus,
rituximab is now considered a treatment option for refractory cases. However, ischemic organ damage in acute phase and sequelae are observed. Therefore, early diagnosis and novel
therapy are required.