ObjectiveTo summarize the clinical characteristics of thrombotic thrombocytopenic purpura(TTP) presenting neurological manifestations as initial symptoms and improve the diagnostic level. MethodsClinical data of 3 cases of TTP were retrospective analyzed with literatures review. ResultsFever, microvascular platelet clumping resulting in thrombocytopenia, microangiopathic hemolysis, neurological abnormality, and renal dysfunction were the characteristics of TTP. The initial symptom shown as neurological disfunction was easily misdiagnosed as stroke and intracranial infection. Early plasma exchange therapy should be effective. ConclusionsThe TTP presenting neurological manifestations as initial symptoms is easily misdiagnosed and should be early diagnosed and treated .