LAUSR

Reversible cerebral vasoconstriction syndrome (RCVS) is a relatively newly described neurovascular entity. The clinical outcome is generally benign, but sometimes disabling or lifethreatening. Triggers for this condition are variable with a large proportion of idiopathic causes. Several informative papers had been written on this subject which include proposals for diagnostic criteria, differentiation from other cerebral vasculopathies, and imaging features. However, the pathophysiology of the condition is still not well understood, especially in the large proportion of idiopathic cases. A leading hypothesis for the propagation of the condition attributes a significant role to vascular autoregulation disruption similarly to posterior reversible encephalopathy syndrome (PRES) but with different triggers. Early markers of the condition are needed, which would allow prompt treatment, avoid unnecessary studies and shed some light on the RCVS mechanism. Recently a salient study of 23 RCVS patients in South Korea described a phenomenon of contrast enhanced fluid attenuation inversion recovery (CE FLAIR) magnetic resonance imaging (MRI) hyperintensity in cortical sulci interpreted as blood brain barrier (BBB) disruption and showed its correlation to clinical outcome. We observed similar findings in Israeli population, and 18 out of 21 confirmed RCVS patients had exclusively posterior sulcal contrast enhancement (in the posterior occipital, parietal or cerebellar sulci) on CE FLAIR sequences (Figure 1). We also found a positive correlation between the extent of the CE FLAIR involvement and RCVS severity defined by a composite outcome score calculated for each individual patient. We graded the severity of RCVS by a composite neurological score that included PRES like edema appearance on MRI (0, 1), clinical seizures (0, 1), subarachnoid hemorrhage (0, 1), brain ischemia (0, 1) and thunderclap headache on initial presentation (0, 1). Multivariate logistic regression analysis was used to assure that the score components were not affected by demographic or clinical variables. The score was devised according to previously described markers of RCVS severity. The grading of CE FLAIR included the composite of intensity of sulci enhancement by contrast (0, no signal; 1, for mild signal; 2, for substantial signal) with its distribution throughout the brain (1 point for each involved lobe—including cerebellar hemispheres; 0–10). All the patients were female with a median age of 41, 17 (68%) with a non-significant prior medical history. Twenty-three patients (92%) were considered for an analysis (with available of MRI scans). Finally, 21 (85%) confirmed RCVS patients were included for the analysis. None of the patients exhibited a cellular inflammatory reaction in the CSF (Supplementary materials). All Early Fluid Attenuation Inversion Recovery Sulcal Contrast Enhancement Correlates with Severity of Reversible Cerebral Vasoconstriction Syndrome