Reversible Cerebral Vasoconstriction Syndrome Treated with Plasmapheresis and Calcium Channel Blocku

Objective To describe a case of a treatment refractory cerebral medium vessel arteriopathy, likely representing a severe case of reversible cerebral vasoconstriction syndrome (RCVS). Background RCVS is a cerebrovascular dysregulation syndrome typically characterized by acute, segmental cerebral arterial narrowing. The most common presenting symptom is acute onset thunderclap headache. The term “reversible” refers to reversibility of angiopathic abnormalities; however, the cerebral vasoconstriction is not always reversed. This case highlights a severe case of RCVS with unclear etiology and novel treatment approach. Design/Methods N/A Results Patient is a 55‐year old female who presented to the ED with acute onset of left‐sided numbness. Her initial workup included CT angiography of the neck which demonstrated an unstable thrombus in the distal right common carotid artery and high‐grade stenosis of the right carotid bulb. She was initiated on intravenous heparin for the unstable thrombus in addition to aspirin 81 mg daily. The patient underwent carotid endarterectomy (CEA) with vascular surgery 2 days after admission. She was discharged the following day on aspirin 81 mg daily as antiplatelet monotherapy. She presented again 11 days following discharge with acute onset of left leg weakness and recurrent, acute onset thunderclap headache. CT angiogram head and neck demonstrated multifocal high‐grade stenosis and occlusions along the right MCA and bilateral ACAs, suspicious for RCVS. Pertinent medications at the time of admission included tramadol (recently initiated for new headache) and nicotine patch; the patient denied any additional changes in medications including use of serotonergic agents. The patient was initiated on a non‐dihydropyridine calcium channel blocker with verapamil 40 mg three times daily and discharged 5 days after admission with resolution of her symptoms. The patient presented again to the ED the following day with acute onset of post‐coital left leg weakness. Initial imaging was significant for interval worsening of previously demonstrated vasospasms of the right MCA and bilateral ACAs. The patient was ultimately admitted for over one month, with multiple treatments attempted based on the refractory nature of her disease. With concern for an autoimmune component to her focal arteriopathy, considered potentially secondary to a hypersensitivity reaction to the bovine graft in CEA, she was initiated on intravenous methylprednisolone. Intravenous or oral corticosteroids were continued throughout admission. The patient also underwent multiple angiographies with intraarterial administration of verapamil, milrinone, and nicardipine, with only transient improvement in vasoconstriction following each treatment. Extensive serum and cerebrospinal fluid testing failed to demonstrate a causative etiology for her refractory symptoms. 3 weeks into admission, the patient was initiated on plasmapheresis for five days. Her exam stabilized, and she was successfully discharged to a long term acute care facility. Conclusions Although typically a self‐limited condition, RCVS can be treatment‐refractory and result in irreversible changes in cerebral function. Our case demonstrates a severe, refractory multifocal cerebral vasoconstriction syndrome, resistant to multiple therapies, which ultimately responded to treatment with plasmapheresis.