Abstract | November 9, 2021
A Rare Case of Reversible Cerebral Vasoconstriction Syndrome in a Patient with Systemic Sclerosis
Learning Objectives
- With a typical thunderclap headache patient, it is necessary to consider RCVS as a differential diagnosis from systemic sclerosis-related migraine and vasculitis.
- The clinical presentation of RCVS is recurrent sudden, severe thunderclap headaches. The patient can be with or without focal neurologic deficits. The diagnosis of RCVS is based on the recurrent thunderclap headache and typical brain imaging findings which is reversible multifocal segmental narrowing of the cerebral arteries.
Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disease and increasingly recognized. This syndrome is represented by recurrent severe thunderclap headache, with or without neurological symptoms, the typical brain image test will show reversible diffuse segmental vasoconstriction of the cerebral arteries. There are some case reports of RCVS related to SLE and immunosuppressant therapy. But to our knowledge, there is very limited report of RCVS in systemic sclerosis patients. We report a case of RCVS in a systemic sclerosis patient with typical radiological change and good response to the treatment.
Case Presentation: A 44-year-old female, present in the hospital due to body aches, decreased appetite, Raynaud phenomenon, fingertip pain and ulcerations, facial and back rash, skin tightness, and skin depigmentation. Positive with anti-Scl-70 and ANA antibody. The patient was diagnosed with systemic sclerosis and was treated with steroids, mycophenolate mofetil, and hydroxychloroquine. During the treatment, the patient developed a severe thunderclap headache with left lower extremity weakness. The Head CTA showed multifocal long segment stenosis ranging from mild to moderate in severity involving the M2 segments of the right MCA. The differential diagnosis including migraine-related to scleroderma, vasculitis, and RCVS.
Final/Working Diagnosis: The brain angiogram showed diffused mild and modest smooth stenoses in the cerebral arteries, the stenoses responded to intra-arterial calcium channel blocker and consistent with findings of reversible cerebral vasoconstriction syndrome.
Management/Outcome/and or Follow-up: Consider patient ‘s headache and lower extremity weakness is due to RCVS. Gave patient nimodipine 60mg QD, patient’s headache resolved, left lower extremity weakness improving. The patient was discharged with nimodipine, follow-up outpatient. Until now, the patient’s headache is under controlled, no recurrent.
