Headache Medicine 2020, 11(2):32-34 ISSN 2178-7468, e-ISSN 2763-6178

ASAA

DOI: 10.48208/HeadacheMed.2020.10

Headache Medicine

Clinical Correspondence

Vessel wall imaging for diagnosis and follow-up of basilar artery

reversible cerebral vasoconstriction syndrome (RCVS)

Matheus Kahakura Franco Pedro Paulo Sérgio Faro Santos Bruno Augusto Telles

Department of Interventional Neuroradiology, Instituto de Neurologia de Curitiba, Curitiba, Paraná, Brazil.

Abstract

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a clinical and radiological syndrome

that is primarily dened by thunderclap headache, with or without further neurological decits,

and segmental intracranial vasoconstriction that resolves within three months. The current no-

menclature was only established in 2007, but it has been known with diferent names for over

fty years. The pathophysiology, while still not completely understood, seems to point towards

a disease based on abnormalities of vascular tonus without structural inammation. It is clear,

however, that patients with RCVS often have triggers, especially drugs or other vasoactive subs-

tances. Distinguishing this entity from others, especially subarachnoid hemorrhage and arterial

dissection, is extremely important, given the particular prognosis and need of immediate treatment

of each disease. The preferred imaging method has long been the angiography; however, new

magnetic resonance imaging (MRI) such as vessel wall imaging have allowed for non-invasive

diagnosis and follow-up. The authors report a case in which MRI was used in a patient with

basilar artery RCVS and present a literature review.

Matheus Kahakura Franco Pedro

matheuskfpedro@hotmail.com

Edited by

Marcelo Moraes Valença

Mario Fernando Prieto Peres

Received: April 2, 2020.

Accepted: April 27, 2020.

Keywords:

Vasoconstriction

Magnetic Resonance Imaging

Vascular Headaches

ASAA

Pedro MKF, Santos PSF, Telles BA.

Vessel wall imaging for diagnosis and follow-up of basilar artery reversible cerebral vasoconstriction syndrome (RCVS)

Introduction

eversible Cerebral Vasoconstriction Syndrome (RCVS) is a cli-

nical and radiological nosologic entity that is primarily dened

by hyperacute onset of thunderclap headache and segmental

intracranial vasoconstriction that resolves within three months, with

or without further neurological decits

. Though digital subtraction

angiography has long been the standard work-up exam, the role

of magnetic resonance imaging, particularly after the renement of

vessel wall imaging, has substantially expanded

2,3

Case Report

T h e a u t h o r s p r e s e n t t h e c a s e o f a 3 1 y e a r s - o l d C a u c a s i a n

female with no previous history of headache, who presented to the

ER due to a sudden, thunderclap occipital headache while per-

forming strenuous physical activity (cross-t). No other neurolog-

ical symptoms or decits took place. She underwent an arterial

angiotomography which suggested vascular lumen reduction of

the basilar artery. Her laboratory work-up showed no noteworthy

alteration. Afterwards, she underwent brain MRI with vessel wall

imaging on a 3-Tesla machine, which conrmed a stenosis inferior

to 50% on the middle section of the basilar artery along with gad-

olinium enhancement towards the vertebro-basilar junction (Figure

1). Her headache receded without need for medication and she

was released for outpatient follow-up. After three months without

any symptom, another brain MRI with vessel wall imaging on the

same machine was performed, showing near complete resolution

of the stenosis, as well no further enhancement on the basilar artery

after gadolinium injection (Figure 2). On the same outpatient visit,

the patient reported regular use of a performance enhancement

compound including caffeine and bupropion.

Figure 1. Left - coronal view of reconstruction of arterial angioMRI on a 3

Tesla magnet conrming stenosis of the middle third of the basilar artery

(arrow); right - coronal slice of vessel wall imaging after gadolinium in-

jection, with impregnation of the basilar artery near the vertebro-basilar

junction (arrow).

Figure 2. Left - coronal view of reconstruction of control arterial angioMRI

after twelve weeks showing near complete resolution of the stenosis; right

- coronal slice of control vessel wall imaging after gadolinium injection

showing complete resolution of the gadolinium enhancement by the verte-

bro-basilar junction.

Discussion

Though this nosologic entity was rst reported over fty years

ago, its most consistent description came in 1988 by Call and

Fleming

; Calabrese proposed the current nomenclature in 2007

and established formal diagnostic criteria, thus unifying the many

“diseases” with similar clinical and radiologic features under a single

term.

No precise data on incidence is currently available, though

it doesn’t appear to be particularly rare

. The pathophysiology

remains a mystery, although alteration on vascular tone leading

to vasoconstriction seems to be the main mechanism

, which

is supported by the lack of vascular or perivascular histological

abnormalities on biopsy of brain tissue. The role of sympathomimetic

vasoactive substances is well known, with caffeine and bupropion

having been previously recognized as triggers

7,8

. The differential

diagnosis includes subarachnoid hemorrhage, cervical arterial

dissection, and primary angiitis of the central nervous system; as

such, correct differentiation between these entities is of paramount

importance, given the different mechanisms and treatments. In

terms of prognosis, the disease is monophasic and typically self-

limiting, with the criteria establishing resolution within three months.

Conclusion

This case illustrates the typical course of the disease and the need

to recognize it and differentiate from other vascular diseases of the

central nervous system. The use of MRI with vessel wall imaging

allows for both accurate diagnosis and follow-up in a non-invasive

manner.

ASAA

Pedro MKF, Santos PSF, Telles BA.

Vessel wall imaging for diagnosis and follow-up of basilar artery reversible cerebral vasoconstriction syndrome (RCVS)

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