AUTHOR=Breville Gautier , Bailly Amelie , Fisch Loraine , Kulcsar Zsolt , Pugin Deborah , Carrera Emmanuel 

TITLE=Case Report and Review of the Literature: Fatal Reversible Cerebral Vasoconstriction Syndrome

JOURNAL=Frontiers in Neurology

VOLUME=12

YEAR=2021

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2021.589062

DOI=10.3389/fneur.2021.589062

ISSN=1664-2295

ABSTRACT=<p><bold>Background:</bold> A fatal outcome occurs in 2% of patients with Reversible Cerebral Vasoconstriction Syndrome (RCVS). Due to its rarity, guidelines for the management of the most severe forms of RCVS are lacking.</p><p><bold>Case presentation:</bold> Here, we describe the case of a 55 year-old woman who died from complications of RCVS and reviewed patients with fatal outcome reported in the literature. In our patient, the first episode of neurological deterioration was preceded by an increase of cerebral blood flow velocities assessed with transcranial Doppler. A fatal evolution could not be prevented despite therapeutic escalation consisting of multiple non-invasive and invasive treatments including cervical sympathetic bloc and continuous arterial infusion of nimodipine at the site of severe vasoconstriction.</p><p><bold>Conclusion:</bold> This case and the review of literature illustrate the challenges in the management of patients with severe RCVS. We describe here how monitoring of cerebral blood flow might help anticipate clinical worsening at the beginning of the disease and propose novel invasive and non-invasive therapeutic strategies based on monitoring of neurophysiological parameters.</p>