Central nervous system involvement in thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an uncommon multisystem diseas e characterized by microangiopathic hemolytic anemia, thrombocytopenic purp ura, fluctuating neurologic dysfunctions, renal disease, and fever. Delayed diagnosis and treatment may lead to a high mortality rate that can be as g reat as 90%. We describe 7 patients in whom CNS involvement was the first c linical manifestation of TTP. Despite early diagnosis and treatment, 3 pati ents died because of multiple system organ failure. Four patients recovered completely. In patients with focal neurologic disorders in whom infectious CNS diseases were excluded TTP should be considered in the differential di agnosis.