Large vessel stroke as initial presentation of thrombotic thrombocytopenic purpura

Abstract

A 67-year-old right-handed woman presented with dysarthria, left upper extremity weakness and right-sided neglect of 3 hours duration. Imaging of the brain revealed acute right middle cerebral artery stroke; however, tissue plasminogen activator could not be administered due to severe thrombocytopenia. A peripheral smear revealed schistocytes and the patient was treated empirically for thrombotic thrombocytopenic purpura (TTP) with therapeutic plasma exchange. An extensive workup revealed no embolic source or other cause for stroke, and a diagnosis of large vessel infarct secondary to TTP was made. After a prolonged hospital course, the patient had partial neurological recovery and was discharged to a rehabilitation facility. Although transient neurologic deficits due to small vessel occlusions are well described in TTP, large vessel infarct can occur as well. This diagnosis should be considered in patients presenting with concomitant stroke and thrombocytopenia, as untreated TTP is nearly always fatal.

Identification of Biomarkers in Patients with Thrombotic Thrombocytopenic Purpura Presenting with Large and Small Ischemic Stroke

“…A reduction of plasma ADAMTS13 activity leads to an accumulation of highly adhesive ultra-large von Willebrand factor multimers on endothelial surface [ 9 , 10 ] and in circulation [ 11 , 12 , 13 ]. This ultra-large von Willebrand factor captures flowing platelets in circulation, resulting in excessive thrombus formation in areas of endothelial activation and/or injury [ 14 , 15 , 16 ]. If left unrecognized or untreated, TTP carries an exceedingly high mortality (90–95%) [ 1 , 2 , 17 ].…”

“…If left unrecognized or untreated, TTP carries an exceedingly high mortality (90–95%) [ 1 , 2 , 17 ]. Therapeutic plasma exchange (TPE) or plasma infusion has dramatically reduced mortality and morbidity rates [ 2 , 14 , 17 ]. Recent advances in therapeutic strategy including the use of rituximab [ 18 , 19 , 20 ] and caplacizumab [ 21 , 22 , 23 ] have further improved survival rate and long-term outcome.…”

“…The most common symptoms include fluctuating mental status or persistent encephalopathy, aphasia, paresthesia, motor deficits, and seizure [ 27 ]. The presumptive mechanism is from thrombi in small vessels that lead to transient occlusion or small infarcts [ 14 ]. The typical small ischemic strokes seen in TTP are thought to be largely reversible in most patients if they survive the systemic issues related to TTP [ 25 ].…”

Congenital thrombotic thrombocytopenic purpura presenting in adulthood with recurrent cerebrovascular events

“…These cases suggest that, in patients with unexplained stroke and an absence of other risk factors, TTP should be considered, even in the absence of typical haematological derangement 5–9…”

“…While neurological involvement including transient neurological deficit, is well-recognised in TTP due to occlusion of small vessels, large vessel occlusion is less typical, although has been previously recognised 5. Sugarman et al report a case of a 67-year-old patient presenting with right MCA territory infarct, in whom no embolic source was found, but in whom thrombocytopenia and presence of schistocytes led to a diagnosis of TTP, complicated by large vessel occlusion 5…”