Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Apr 21;14(4):e24364.
doi: 10.7759/cureus.24364. eCollection 2022 Apr.

A Case Report of Reversible Cerebral Vasoconstriction Syndrome in a Patient With Systemic Scleroderma

Jieying Liu  1 , Mengni Guo  1 , Richard D Beegle  2 , Ruoyu Miao  3 , Manoucher Manoucheri  1
Affiliations
Case Reports

A Case Report of Reversible Cerebral Vasoconstriction Syndrome in a Patient With Systemic Scleroderma

Jieying Liu et al. Cureus. .

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is represented by recurrent severe thunderclap headache, with or without neurological symptoms. RCVS can be primary or secondary to several factors. Here, we present a case of RCVS in a patient with systemic scleroderma. A 44-year-old female patient presented to the hospital due to Raynaud's phenomenon, fingertip pain ulceration, skin tightness, and skin depigmentation. She was diagnosed with systemic scleroderma. After four days of steroids, immunosuppressants (mycophenolate mofetil), and hydroxychloroquine, the patient developed severe thunderclap headaches and left lower extremity weakness. The computed tomography angiography (CTA) showed multifocal segmental vasoconstriction of the cerebral arteries. The patient's headache and body weakness resolved after starting an oral calcium channel blocker (nimodipine).

Keywords: calcium channel blocker; immunosuppressants; reversible cerebral vasoconstriction syndrome; steroid; systemic scleroderma.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Rash on the patient’s back
Figure 2
Figure 2. Axial and sagittal maximum intensity projection (MIP) post-contrast CTA images of the head
Axial (A) and sagittal (B) maximum intensity projection (MIP) post-contrast CTA images of the head demonstrate a narrowed posterior division M2 branch of the right MCA (white arrows). There is a sharp transition with reconstitution of the normal caliber of the vessel distally (red arrows).
Figure 3
Figure 3. Axial and coronal MIP post-contrast CTA image of the head
A: Axial MIP post-contrast CTA image of the head demonstrates a focal narrowing of the A1 segment of the right ACA (white arrow). B: Coronal MIP post-contrast CTA image of the head demonstrates focal narrowing of the P1 segment of the right PCA (white arrow) and focal narrowing of the proximal left superior cerebellar artery (red arrow).
See this image and copyright information in PMC

References

    1. Reversible cerebral vasoconstriction syndrome: recognition and treatment. Cappelen-Smith C, Calic Z, Cordato D. Curr Treat Options Neurol. 2017;19:21. - PubMed
    1. Reversible cerebral vasoconstriction syndrome. Calic Z, Cappelen-Smith C, Zagami AS. Intern Med J. 2015;45:599–608. - PubMed
    1. Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome: clinical and radiological considerations. Pilato F, Distefano M, Calandrelli R. Front Neurol. 2020;11:34. - PMC - PubMed
    1. Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus. Ashraf VV, Bhasi R, Ramakrishnan KG, Praveenkumar R, Girija AS. Neurol India. 2012;60:635–637. - PubMed
    1. A systemic lupus erythematosus patient with thunderclap headache: reversible cerebral vasoconstriction syndrome. Chung SW, Lee KM, Heo SH, et al. Lupus. 2019;28:898–902. - PubMed

Publication types

LinkOut - more resources