However, the article raises many important questions and should stimulate further work, ideally collaboratively at multiple centers to increase study power and generalizability, on this fascinating and enigmatic clinical syndrome. Reversible cerebral vasoconstriction syndrome (RCVS) represents a potentially under-recognized cause of thunderclap headache in patients presenting to the ED. On May 25, 2018, the GDPR (General Data Protection Regulation) went into effect and changed how organizations deal with personal data of customers located in the EU. Multiple etiologies for RCVS are suggested by the many and varied associations, including sympathomimetic, serotonergic, or other drugs, pregnancy, and the puerperium,2 although no obvious cause can be found in approximately one-half of cases. Figure 1 highlights the area of vasoconstriction seen on CTA that was diagnostic in this case. As a result of historical features overlapping the presentation of SAH, particularly the rapidity of onset and peak of severity, these patients are typically investigated for SAH and when that workup ultimately is reassuring, clinicians may often misattribute RCVS symptoms as migrainous. Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D: Valença MM, Andrade-Valença LPA, Bordini CA, Speciali JG: Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. In the majority of cases, there exists an identifiable trigger such as exertion, coughing, defecation, or the use of vasoactive substances [3-4]. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See Table 1 for a detailed timeline. David Werring is supported by a Department of Health and Higher Educational and Funding Council for England Clinical Senior Lectureship Award. These include thunderclap headache with vasospasm, migrainous vasospasm (or “crash” migraine), migrainous angiitis, drug-induced angiitis, postpartum angiopathy, and benign angiopathy of the central nervous system. On the fourth ED visit a CT angiogram (CTA) was completed that demonstrated the characteristic “string of beads” appearance of the middle cerebral artery (MCA) diagnostic of RCVS. "Never doubt that a small group of thoughtful, committed citizens can change the world. Common migraine treatments such as triptans may worsen symptoms, and in some cases have been documented as inducing RCVS [13]. It may well be that therapy needs to be tailored to the underlying causes or mechanisms, although current approaches focus on the shared common factor of vasospasm, which logically might reduce the main complications of infarction and hemorrhage. The authors found that cortical subarachnoid hemorrhage was the most common pattern, affecting 27 of the 40 cases of hemorrhage; women and those with migraine were at highest risk. Finally, at his fourth presentation (nine days since initial onset), his progressive headache remained typical for a SAH and was not in keeping with a post-lumbar puncture headache. All registered users are invited to contribute to the SIQ™ of any published article. His Glasgow Coma Scale (GCS) score was 15/15. The college also refused to reveal the cost of the investigation, but it is expected to run into tens of thousands of pounds. Reviewing with Cureus is easy, fast and hassle-free! In a recent review about fatal causes of RCVS, a good prognosis was found in 78–90% of patients with RCVS, but a mortality rate of 1–5% mainly occurred in postpartum and pregnancy. However, in the present study, vasospasm was concomitant with intracerebral hemorrhage in 15, and after hemorrhage in 14 patients; the mean time of diagnosis of intracranial hemorrhage was 2.2 days from headache onset and the mean time to diagnosis of vasoconstriction was 6.6 days. Reversible cerebral vasoconstriction syndrome remains an underdiagnosed presentation of thunderclap headache to the ED, but is an important consideration in cases where SAH has been ruled out. No acute infarct was evident. Table 1 illustrates the sequence of presentation and investigations the patient received. While the full details surrounding this incident could not be recounted by the patient, it is possible that this positive family history was also contributory to our patient's presentation. As RCVS presents similarly and with equal frequency to SAH [9], it is important to consider it during the early stages of workup for SAH in the ED. Future studies should explore whether patients with a family history of thunderclap headache are at higher risk of RCVS. By continuing to browse this site you are agreeing to our use of cookies. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. A lumbar puncture was performed on the third visit to rule out meningitis. Conclusion RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features. Abstract Reversible cerebral vasoconstriction syndrome (RCVS) represents a potentially under-recognized cause of thunderclap headache in patients presenting to the ED. A lumbar puncture performed on his third presentation the following day (seven days since symptom onset) was unremarkable. RCVS typically presents as a sudden onset, excruciating headache that may be associated with nausea, vomiting, … He had no gait abnormalities or cerebellar signs. Recurrent thunderclap headaches, seizures, strokes, and non-aneurysmal subarachnoid haemorrhage can all reveal reversible cerebral vasoconstriction syndrome. Roberts A, Sowers N (May 31, 2020) A Case of Reversible Cerebral Vasoconstriction Syndrome in a Healthy Adult Male. Contact Us, Correspondence to Dr David Werring, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Box 6, Queen Square, London WC1N 3BG, UK. On this occasion, he received metoclopramide, ketorolac, and acetaminophen with codeine for pain management. The label RCVS is an attempt to unify these various descriptions of a distinct clinico-radiological pattern. His neurological exam was unremarkable, with no apparent cranial nerve abnormalities. The opinions expressed in this editorial are not necessarily those of the editors or of the American Heart Association. He described a paternal history of a SAH leading to a generalized seizure several years ago. Although RCVS generally resolves without significant sequelae, a rare and possibly underrecognized hemorrhagic presentation has a worse potential … This can only be resolved by reliably predicting a diagnosis of RCVS without waiting for reversibility to be proven, which currently is not possible. An unenhanced head CT was performed approximately 24 hours after symptom onset, which revealed no significant findings. Further documentation of cases in younger males is important to determine whether the features of RCVS differ by gender. Although recruitment in such a specialist referral center is not free of bias, and the diagnostic work-up was not standardized, the findings do suggest that intracranial hemorrhage in RCVS is much more common than previously thought and further challenges the concept of RCVS as a benign self-limiting syndrome.2. In the setting of a firm diagnosis of RCVS, some advocate treatment with a calcium channel blocker either alone or combined with a faster tapering course of prednisone than would be used in PACNS. Although this patient had some historical features that are documented to be associated with RCVS (e.g., vasoactive substance use, migraine), it is not clear whether they were temporally related to the headache. 1-800-242-8721 RCVS is a syndrome still in the process of being defined; our understanding and knowledge are increasing as neuroimaging methods improve and more cases are discovered. While all registered Cureus users can rate any published article, the opinion of domain experts is weighted appreciably more than that of non-specialists. Scholarly Impact Quotient™ (SIQ™) is our unique post-publication peer review rating process. Reversible cerebral vasoconstriction syndrome is more common in women, typically in their 40s-50s who have a history of migraine [4-5]. rcvs, reversible cerebral vasoconstriction syndrome, emergency department, Cite this article as: It remains to be seen if the review of ‘under my care’ and 24-hour care will be the profession’s own moment of self-inflicted and long-lasting harm. The features of what is now called RCVS have been repeatedly recognized, especially since an influential early report by Drs Call and Fleming et al1 published in 1988.

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