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Table 1 Summary of potential pathophysiology of RCVS

From: Pathophysiology of reversible cerebral vasoconstriction syndrome

Key elements

Main mechanism

Strength of evidence

Possible treatment strategiesa

Dysregulation of cerebral vascular tone

• Suddenly increased sympathetic drive

• Passive autoregulatory response against blood pressure surge

• Consequences of endothelial dysfunction, oxidative stress, and BBB disruption

• Dysregulated circulating miRNAs associated with vasomotor regulation

+++

• Early identification and avoidance of triggers or secondary causes with heightened sympathetic drive

• Cerebrovascular-selective calcium channel blockers?

Sympathetic overactivity

• Predisposing sympatho-vagal imbalance

• Triggers or secondary causes associated with heightened sympathetic drive

++

• Early identification and avoidance of triggers or secondary causes

Endothelial dysfunction

• Impaired endothelial repairing capacity (i.e., reduced endothelial progenitor cells)

• Systemic endotheliopathy caused by secondary causes

+++

• Avoidance or early removal of secondary causes

Excessive oxidative stress

• Increased reactive oxygen species and lipid peroxidation upon increased shearing stress and endothelial dysfunction

++

• Antioxidants?

Blood–brain barrier disruption

• Excessive blood pressure surge and pulsatile flow exceeding autoregulatory capacity

• Oxidative stress

• Circulating microRNAs (miR-130a)

+++

• Avoid blood pressure surge

• Antioxidants?

Altered trigeminovascular nociception

• Sudden stretch of perivascular trigeminal nociceptors (by dilatation of distal arteriole or meningeal artery)

• Increased BBB permeability

• Altered circulating microRNAs

?

• The cerebrovascular-selective calcium channel blocker nimodipine

• CGRP-targeting therapy? (with risk of vasoconstriction)

Genetic predisposition

• Genetic contribution to disease vulnerability

+

• Gene-based mechanism targeting therapy?

Sex hormones

• Hormonal modulation of cerebral vascular tone

+

• Avoid abrupt hormonal fluctuation in vulnerable subjects?

Inflammation

• Systemic or perivascular inflammation causing endothelial dysfunction

+

• Anti-inflammatory therapy? (Of note, steroid may be associated with poor prognosis)

  1. Strength of evidence: +++: supported by multiple studies from different research groups; ++: supported by at least one study from single research group; +: based only on clinical observations; ?: based only on hypothesis
  2. aTreatment without direct supporting evidence is ended with a question mark