Reversible cerebral vasoconstriction syndrome in a background of eclampsia responding to milrinone infusion

Abstract

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare neurological disorder with a complex physiopathology that is not fully understood. Suggested underlying mechanisms include failure of autoregulation, endothelial dysfunction, and oxidative stress. It is characterized by reversible multifocal constriction of the cerebral arteries, and can be triggered by many conditions, including, vasoactive medications (eg, triptans), cerebrovascular events, primary headache disorders, and metabolic causes (eg, hypercalcemia). RCVS can also be associated with pregnancy-related conditions, such as thrombotic thrombocytopenic purpura, eclampsia, and pre-eclampsia. Thunderclap headache is the most common clinical manifestation; however, other symptoms can result from complications of the disease, such as stroke, brain edema, and seizures. Several case reports have been published of an association between RCVS and eclampsia, but to the best of our knowledge, only 3 cases were successfully treated with intravenous milrinone and this is the only patient reported in Saudi Arabia. CASE REPORT: We report a case of 25-year-old primigravida woman who presented with acute-onset headache, nausea, elevated blood pressure, and generalized tonic clonic seizure. She was diagnosed as having RCVS secondary to eclampsia based on clinical and radiological features. She was initially started on nimodipine, which is usually the first-line management of RCVS, as well as magnesium sulfate and levetiracetam; however, she only achieved full recovery after starting intravenous milrinone. CONCLUSIONS: Milrinone is one of the emerging drugs for treatment of RCVS, and this case report delineates the potential of using the drug, especially in cases refractory to standard therapy.