Moschcowitz syndrome mimicking basilar pathology

Background: Thrombotic thrombocytopenic purpura (TTP) is a clinically heterogeneous syndrome first described by Moschkowitz in 1924. Often postinfectious, but also autoimmune- or drug-associated, TTP is characterised by thrombocytopenia, Coombs-negative haemolytic anaemia, neurological deficits, renal impairment and fever. Inhibitory antibodies against von Willebrand factor (vWf)-cleaving protease lead to a functional deficiency of this protease and to unusually large multimers of vWf. These multimers are capable of agglutinating circulating platelets, thereby reducing platelet count and causing thrombotic micro…