Background: Refractory condition can occur in 10-30% of all cases of thrombotic thrombocytopenic purpura despite increased frequency of total plasma exchange. Rituximab can affect the clinical outcome of the refractory cases. However, little is known about usefulness of rituximab on central nervous system involvement mimicking acute ischemic stroke.
Methods: We report the case of a woman with refractory thrombotic thrombocytopenic purpura who developed an acute onset right sided paralysis, dysarthria, and central facial paralysis, suggestive of cerebrovascular accident while under plasma exchange, corticosteroid, and vincristine therapy.
Results: After initiation of rituximab (375 mg/m(2) weekly for 4 weeks), a dramatic response occurred and the patient's neurologic function recovered fully within days. Sustained remission was achieved, and the patient was well 1 year after her admission, while she was on azathioprine treatment.
Conclusion: This report suggests that rituximab can provide a good outcome of the dramatic central nervous system involvement in patients with thrombotic thrombocytopenic purpura.