TTP is an uncommon and potentially deadly illness, with >90% mortality if left untreated; ADAMTS13 deficiency causes extensive microvascular thromboses and organ harm, particularly in organs with high shear stress, such as the brain. Doctors have traditionally feared acute neurological problems, and have been associated with a poor prognosis. TTP, on the other hand, was no longer viewed as two extremes of acute presentation and remission but rather as a chronic illness with the potential for long-term symptom burden once recognized. There was no agreement on optimal neuroimaging timing and modality. As researchers learned more about the alterations found throughout the acute and chronic stages of TTP, there was potential for neuroimaging to play a larger role in guiding care and secondary prevention of vascular disease. 

Reduced ADAMTS13 activity had been linked to an increased risk of thrombosis, and new treatments such as caplacizumab and recombinant ADAMTS13 might provide neuroprotection. Given the growing evidence of neurocognitive and psychiatric illness, the need for screening and prompt intervention in TTP should not be underestimated. As more patients survive their first TTP presentation, it is more important to learn more about the long-term morbidity that these patients face.

Reference:onlinelibrary.wiley.com/doi/10.1111/bjh.18127

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