Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is one of the most important differential diagnosis in patients with thunderclap headache (TCH). We aimed to develop a new scoring system for RCVS in patients with TCH. We retrospectively analyzed 72 patients enrolled in the prospective study of TCH conducted in 2015–2016 (derivation set). We identified possible predictors for the diagnosis of RCVS and constructed a prediction model (RCVS–TCH score) using the multivariable logistic regression model. Diagnostic performance was validated to an independent validation set from our headache registry. The derivation set comprised 41 patients with RCVS and 31 with non-RCVS, and the validation set included 253 patients with TCH (165 with RCVS and 88 with non-RCVS). The RCVS–TCH score (range: 0–12) contained four predictors: recurrent TCHs, female sex, triggering factor for TCH (single or multi) and blood pressure surge. The C-index of RCVS–TCH score was 0.929 (95% CI = 0.874–0.984). The RCVS–TCH score ≥ 7 had a sensitivity of 80% and a specificity of 97% in discriminating RCVS from non-RCVS. In the validation set, RCVS–TCH score showed a C-index of 0.861 (95% CI = 0.815–0.908). In our study, the RCVS–TCH showed good performance, which may aid the diagnosis of RCVS among patients with TCH.

Highlights

  • Reversible cerebral vasoconstriction syndrome (RCVS) is one of the most important differential diagnosis in patients with thunderclap headache (TCH)

  • The ­RCVS2 score would not be useful for the differential diagnosis of TCH, and any patients with TCH can be falsely classified as having RCVS using this score

  • We validated the performance of our prediction model and compared it with the R­ CVS2 score in unselected patients with TCH

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Summary

Introduction

Reversible cerebral vasoconstriction syndrome (RCVS) is one of the most important differential diagnosis in patients with thunderclap headache (TCH). Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical and radiological syndrome characterized by recurrent thunderclap headaches (TCHs) and reversible cerebral vasoconstriction of the cerebral ­arteries[1] It is one of the most important differential diagnosis in patients with TCH because a substantial proportion of patients with RCVS can have neurological complications such as ischemic stroke, cortical subarachnoid hemorrhage (SAH), intracerebral hemorrhage, and posterior reversible encephalopathy syndrome (PRES)[2,3,4,5,6]. The score includes clinical and imaging features such as recurrent/single thunderclap headache, vasoconstrictive trigger, sex, SAH, and carotid artery involvement. This score showed excellent performance in distinguishing between RCVS and intracranial vasculopathies. We validated the performance of our prediction model and compared it with the R­ CVS2 score in unselected patients with TCH

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