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Background Thrombotic thrombocytopenic purpura (TTP) is a rare haematological disorder characterised by disseminated microvascular thrombosis due to reduced ADAMTS-13 activity. Neurological manifestations of TTP are common, including seizures, headaches, visual disturbances and cerebrovascular events (CVE). Cardiovascular risk factors accelerate atherosclerosis, increasing the risk of CVE and cognitive impairment.Methods A systematic literature review was conducted following the PRISMA protocol. 14 studies reported neurological symptoms at presentation or during the follow-up of treated TTP patients were included. A proportional meta-analysis evaluated demographic and risk factors that may predict neurological manifestations in TTP.Results 14 studies (9235 patients) were included in the systematic review and 13 studies (7047 patients) were included in the proportional meta-analysis. The cumulative prevalence of neurocognitive symptoms at presentation was 45%. Headaches were seen in 37.5%, seizures in 12.1%, stroke in 21.9% and depression in 26.6% of presentations. Silent cerebral infarctions were present in up to 50% of patients in remission.Conclusion Neurocognitive manifestations are common in TTP during acute phases and remission. TTP is associated with a high cardiovascular risk linked to CVE, silent infarcts and cognitive deficits. No established neurological guidelines exist, but the extensive neurological symptoms these patients have make it a condition of interest.smph-west-operational@sheffield.ac.uk