General description

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches, often described as sudden and severe, resembling being struck by lightning. These headaches typically occur bilaterally and affect the entire head. The pain is intense for 1–3 hours, followed by moderate pain that may persist. Episodes recur over 1–4 weeks, with patients experiencing 2–18 thunderclap headaches.

Triggers include physical activities such as postpartum changes, the use of vasoconstrictive agents (e.g., α-adrenergic stimulants, serotonergic drugs). Additional causes include sexual activity, defecation, urination, exercise, coughing, sneezing, emotional stress, illegal drug use (e.g., cocaine, cannabis, amphetamines).

Diagnostic criteria for RCVS include:

1. Acute, severe headaches (often thunderclap headaches), with or without focal neurological symptoms or seizures.
2. Monophasic course, with no new symptoms after one month.
3. Evidence of segmental cerebral arterial vasoconstriction on angiographic studies (catheter angiography, MRA, or CTA).
4. Absence of aneurysmal subarachnoid hemorrhage.
5. Normal or near-normal cerebrospinal fluid findings.
6. Complete or substantial resolution of vasoconstriction within 12 weeks.