Case Presentation: A 40-year-old female patient with a past medical history of hypertension and migraines on amitriptyline presented to the emergency department after noticing a right-sided hemiparesis. She has history of intermittent headaches reaching 10/10 severity within one minute over the last several months and one hemiplegic migraine on the left side. Exam was remarkable for a right facial palsy with decreased sensation to light touch and 2+/5 strength on the right upper and lower extremities. CT head showed ischemic changes of the left inferior lobe and TPA was administered. CT angiography showed diffuse vascular irregularities in multiple territories but no carotid stenosis. Interestingly, the MRI did not reveal acute ischemia. Echo was unremarkable with no obvious embolic source. The patient’s strength recovered to 4/5 with near complete resolution of her facial droop. Two days later, she had worsening right sided strength and a sudden onset headache. No acute changes were seen on repeat imaging. Serologic work-up for vasculitis was negative (HBV, A1c, HIV, ESR, CRP, ANA, SSA, SSB, ANCA, MPO/PR3, syphilis, HCV, SPEP, immunoglobulins). Urine toxicology was negative. Serum electrolytes and liver and renal function tests were within normal limits. She underwent directional coronary atherectomy for evaluation of vasculopathy and was diagnosed with reversible cerebral vasoconstrictive syndrome (RCVS). Verapamil was infused intra-arterially, leading to radiographic and clinical improvement. Serotonin modulating medications, triptans, and NSAIDS were held due to their vasoconstrictive effects.
Discussion: Studies have shown that women with acute transient neurologic events are often misdiagnosed compared to men, indicating missed opportunities for prevention of vascular events amongst women.¹ RCVS is a condition characterized by severe thunderclap headaches with or without other neurologic symptoms. It can present with diffuse segmental narrowing of the cerebral arteries which is reversible within three months. ² The patient’s past diagnosis of a hemiplegic migraine on the left with full recovery may have been more consistent with RCVS. Her history of sudden onset severe headaches and current presentation of right-sided hemiparesis are also consistent with such a diagnosis. RCVS is rare but the current incidence in women is almost four times that of men.³ It can lead to complications of posterior reversible encephalopathy syndrome, seizure, hemorrhage, and brain infarction.⁴ Therefore, it is important to recognize this disease and avoid serotonin modulating medications and acute migraine treatments such as triptans which can aggravate the vasoconstriction associated with RCVS. Calcium channel blockers, including verapamil, can be used to relieve symptoms.
Conclusions: Women with acute neurologic events are more likely to be misdiagnosed compared to men. Despite its name, RCVS can have catastrophic long-term neurologic complications requiring a timely diagnosis and appropriate treatment.