A typical patient with RCVS is a woman between ages 20 and 50 with thunderclap headache, perhaps with a history of migraine. Transient or permanent neurologic signs can develop from RCVS, and hypertension and seizures sometimes occur during the acute phase of the disease.
The pathophysiology of RCVS is unknown, but the condition appears to be associated with a disturbance in cerebrovascular tone. RCVS can occur spontaneously or result from various exogenous or endogenous factors such as catecholamines, serotonin, nitric oxide, prostaglandins, and various inflammatory agents. The immunologic and biochemical factors involved with subarachnoid hemorrhage-associated vasospasm also may be involved with RCVS.
Transfemoral angiography has been considered a requirement for diagnosing RCVS, but “now that we are seeing and recognizing it more, we do not always do catheter angiography because we have excellent CTA imaging,” said Dr. O’Duffy. Another requirement for the diagnosis of RCVS is demonstration that the vascular abnormalities are reversible within 12 weeks from onset. A patient must have nearly normal CSF (eg, protein less than 80 mg/100mL, less than 10 white blood cells/mL, and normal glucose) and no evidence of aneurysmal subarachnoid hemorrhage.
Neurologists commonly treat patients with RCVS with oral calcium-channel blockers. These drugs sometimes are administered intra-arterially to aid the diagnosis by confirming the reversibility of vascular stenosis. Neurologists also try to find and withdraw triggers of RCVS.
In addition to calcium-channel blockers, vasodilators, such as milrinone, and magnesium have been reported as treatments in patients with RCVS. The literature to date suggests that steroids should be avoided, said Dr. O’Duffy.
—Erik Greb