1 - Instituto de Neurologia de Curitiba, Department of Interventional Neuroradiology - Curitiba -Paraná - Brazil.
A Síndrome da Vasoconstrição Cerebral Reversível é uma síndrome clínica e radiológica primariamente definida pela cefaleia em trovoada, tendo ou não outros déficits neurológicos focais, e vasoconstrição intracraniana segmentar, que se resolvem em até três meses. A nomenclatura vigente só se estabeleceu em 2007, embora os aspectos clínicos já sejam conhecidos há mais de cinquenta anos. A fisiopatologia, embora ainda não completamente entendida, sugere uma doença baseada em anormalidades do tônus vascular sem inflamação estrutural. É evidente, no entanto, que pacientes com a síndrome frequentemente tem gatilhos, como drogas ou outras substâncias vasoativas. Distinguir esta entidade de outras, especialmente hemorragia subaracnoide e dissecção arterial, é de suma importância, dados os prognósticos particulares e necessidade de tratamento de cada doença. O método preferido de imagem sempre foi a angiografia; no entanto, novos métodos de ressonância magnética como “vessel wall” permitem diagnóstico e acompanhamento não-invasivos. Os autores relatam um caso em que a ressonância magnética foi utilizada em pacientes com síndrome da vasoconstrição cerebral reversível em artéria basilar e apresentam uma revisão da literatura
Keywords: Imagem por Ressonância Magnética; Vasoconstrição; Cefaleias Vasculares.
Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a clinical and radiological syndrome that is primarily defined by thunderclap headache, with or without further neurological deficits, and segmental intracranial vasoconstriction that resolves within three months. The current nomenclature was only established in 2007, but it has been known with diferent names for over fifty years. The pathophysiology, while still not completely understood, seems to point towards a disease based on abnormalities of vascular tonus without structural inflammation. It is clear, however, that patients with RCVS often have triggers, especially drugs or other vasoactive substances. 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.
Descritores: Vasoconstriction; Magnetic Resonance Imaging; Vascular Headaches.
Reversible Cerebral Vasocon- striction Syndrome (RCVS) is a clini- cal and radiological nosologic entity that is primarily defined by hypera- cute onset of thunderclap headache and segmental intracranial vasocon- striction that resolves within three months, with or without further neurological deficits1. Though digital subtraction angiography has long been the standard work-up exam, the role of magnetic resonance imaging, particularly after the refine- ment of vessel wall imaging, has sub- stantially expanded(2,3).
The authors present the case of AHMA, a 31 years-old Caucasian female with no previous history of headache, who presented to the ER due to a sudden, thunderclap occipital headache while perform- ing strenuous physical activity (cross-fit). No other neurological symptoms or deficits took place. She underwent an arterial angiotomography which suggested vascular lumen reduction of the basilar artery. Her laboratory work-up showed no noteworthy alteration. After- wards, she underwent brain MRI with vessel wall imag- ing on a 3 Tesla machine, which confirmed a stenosis inferior to 50% on the middle section of the basilar artery along with gadolinium enhancement towards the vertebro-basilar junction (Figure 1). Her headache receded without need for medication and she was re- leased 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 af- ter gadolinium injection (Figure 2). On the same out- patient visit, the patient reported regular use of a per- formance enhancement compound including caffeine and bupropion.
Though this nosologic entity was first reported over fifty years ago, its most consistent description came in 1988 by Call and Fleming4; 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 term5. No precise data on incidence is currently available, though it doesn’t appear to be particularly rare6. The pathophysiology remains a mystery, although alteration on vascular tone leading to vasoconstriction seems to be the main mechanism 1, 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 triggers7,8. 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.
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.
- Miller TR, Shivashankar R, Mossa-Basha M, et Reversible cerebral vasoconstriction syndrome, part 1: Epidemiology, pathogenesis, and clinical course. Am J Neuroradiol. 2015;368:1392–9.
- Miller TR, Shivashankar R, Mossa-Basha M, et Reversible cerebral vasoconstriction syndrome, part 2: Diagnostic work-up, imaging evaluation, and diuerential diagnosis. Am J Neuroradiol. 2015;369:1580–8.
- Chen CY, Chen SP, Fuh JL, et Vascular wall imaging in reversible cerebral vasoconstriction syndrome - A 3-T contrast- enhanced MRI study. J Headache Pain. 2018 Aug 30;191.
- Call GK, Fleming MC, Sealfon S, et al. Reversible cerebral segmental vasoconstriction. Stroke. 1988;199:1159–70.
- Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: Reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;1461:34–44.
- Ducros Reversible cerebral vasoconstriction syndrome. Lancet Neurol [Internet].2012;1110:906–17. Available from: http://dx.doi.org/10.1016/S1474-44221270135-7
- Dakay K, McTaggart RA, Jayaraman M , et al. Reversible cerebral vasoconstriction syndrome presenting as an isolated primary intraventricular hemorrhage. Chinese Neurosurg J. 2018;41:1–4.
- Marder CP, Donohue MM, Weinstein JR, et al. Multimodal imaging of reversible cerebral vasoconstriction syndrome: A series of 6 cases. Am J Neuroradiol. 2012;337:1403–10.