RCVS–TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache

DOI: https://doi.org/10.1038/s41598- 021- 87412- 7

Abstract-Summary We aimed to develop a new scoring system for RCVS in patients with TCH.

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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). In the validation set, RCVS–TCH score showed a C-index of 0.861 (95%

CI = 0.815–0.908).

The RCVS–TCH showed good performance, which may aid the diagnosis of

RCVS among patients with TCH.

Extended: The RCVS–TCH score was validated using the validation data set. The RCVS–TCH score showed a superior performance to the RCVS2 score. The RCVS–TCH score can be useful particularly when the angiographic finding

is negative or equivocal.

The RCVS–TCH score should be considered first in the differential diagnosis of thunderclap headache, whereas RCVS2 score would be helpful in the differential diagnosis of intracranial angiopathies.

BP surge was observed in 47% of patients with RCVS, which is similar to the

findings in previous studies [302, 303].

Introduction 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) [302–306]. The RCVS2 score was proposed as a diagnostic tool to distinguish RCVS in

patients with intracranial vasculopathies [307].

The presence of thunderclap headache is the major component of the RCVS2

score, and this alone can lead to the diagnosis of RCVS.

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.

In this study, we aimed to develop a new prediction model for the diagnosis of

RCVS in patients with TCH.

Methods Patients who (1) clearly remembered the mode of onset, (2) reported the time from headache onset to its peak to be < 60 s, and (3) visited within 1 month after the first attack were included, whereas those with (1) aneurysmal SAH, (2) contraindica- tions to magnetic resonance imaging (MRI) or gadolinium enhancement, and (3) clinical manifestations suggestive of infectious meningitis were excluded.

Forty-one patients had RCVS, and 31 had primary TCH or other second-

ary causes.

In the outpatient headache clinic, patients were primarily evaluated using brain MRI and MRA, whereas patients with persistent headaches were referred to the ER and the emergency protocol was then applied.

3.3 Clinical Diagnosis

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For validation, we screened patients from the prospective headache registry of the Samsung Medical Center and extracted 253 patients with TCH who completed neuroimaging work-up within 1 month after onset.

Results When compared with the non-RCVS, female sex, recurrent TCHs, multi-triggers for TCH, and BP surge were more frequently seen in the RCVS group in both datasets.

In the univariable logistic regression analysis of the derivation set, recurrent TCHs (Beta  =  2.31, OR  =  10.08, 95% CI  =  3.37–30.13, p  <  0.001), female sex (Beta = 1.65, OR = 5.18, 95% CI = 1.27–21.19, p = 0.022), the presence of trigger- ing factors for TCH (single, Beta  =  2.37, OR  =  10.68, 95% CI  =  2.62–43.48, p = 0.001; multiple, Beta = 2.85, OR = 17.25, 95% CI = 04.41–67.44, p = 0.001), and BP surge (Beta = 3.25, OR = 25.91, 95% CI = 3.22–208.38, p = 0.002) were associated with RCVS.

Discussions We developed a new prediction model, the RCVS–TCH score, to aid the diagnosis of RCVS in patients with TCH.

The RCVS2 score showed an excellent performance to predict RCVS in patients

with arteriopathies [307].

This is in line with previous studies reporting that 81–90% of patients with RCVS were women, 78–100% had recurrent TCHs, and 75–80% had one or more triggering factors for TCH [302–308].

Only a small proportion of non-RCVS patients with TCH had BP surge, suggest- ing that BP surge may be caused not by a response to severe headache but by the unique pathophysiology of RCVS.

The RCVS–TCH score showed high specificity and sensitivity for discriminat-

ing RCVS in patients with TCH.

This is the first study to develop a prediction model for the diagnosis of RCVS in

patients with TCH.

Conclusions The RCVS–TCH score, a new prediction model for RCVS among patients with TCH, showed good performance in distinguishing RCVS from primary TCH or other secondary causes of TCH.

Our findings would aid the diagnosis of RCVS among patients with TCH when

the aneurysmal subarachnoid hemorrhages were excluded.

Acknowledgement A machine generated summary based on the work of Cho, Soohyun; Lee, Mi Ji; Gil, Young Eun; Chung, Chin-Sang. 2021 in Scientific Reports.

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3.4

Neuroimaging

Machine generated keywords: brain, functional, matter, structural, optic, mri, reti- nal, white matter, restingstate, white, optical, cerebral, cortex, article, functional connectivity

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