Current Understanding of Trousseau’s Syndrome and Therapeutic Considerations

Abstract

It is now realized that cancer patients have a significantly increased risk of cerebral infarction. One of the more common causes of infarction in these patients is acute multiple embolic stroke caused by systemic hypercoagulation known as Trousseau's syndrome (abbreviated as MESTS for the purposes of this review). However, diagnostic procedures for MESTS and the efficacy of anticoagulant therapy have not been established. Some studies have shown that a diagnosis of MESTS requires two findings: (1) evidence of acute cerebral infarction in three major arterial territories in the brain (i.e., the internal carotid arteries and vertebrobasilar artery) simultaneously on diffusion-weighted magnetic resonance images and (2) a significantly increased D-dimer value. Several recent reports indicate that direct oral anticoagulants are effective and safe in the treatment of cancer-associated venous thrombosis, the pathology of which is similar to that of MESTS. When evaluating the indication for direct oral anticoagulants in MESTS, it is important to exclude underlying diseases other than cancer that cause embolic stroke of undetermined source (ESUS) in the general population, which include subclinical paroxysmal atrial fibrillation, paradoxical embolism, artery-to-artery embolism, and vasculitis syndrome. Our previous studies demonstrate that serial investigations of the D-dimer value are very useful for differentiating between Trousseau’s syndrome and other multiple cerebral embolisms, such as atrial fibrillation-derived embolism and so-called ESUS. This review summarizes recent trends in the diagnosis of MESTS as well as treatment, including antithrombotic therapy, and proposes the best algorithm for treatment of MESTS based on the recent literature.