reversible cerebral vasoconstriction syndrome (RCVS)

Last edited 06/2023 and last reviewed 06/2023

Reversible cerebral vasoconstriction syndrome (RCVS) is a relatively recently described condition characterized by sudden, severe ('thunderclap') headache; with or without other acute neurological symptoms and diffuse segmental constriction of cerebral arteries that resolves spontaneously within 3 months (1).

• more than half the cases are seen in the post partum period or after exposure to adrenergic or serotonergic agents (1)
• affects all ages, mean age of onset is 42 years
• affects women more than men (2)

RCVS is thought to be responsible for majority of ' benign thunderclap headaches' (3)

• is the most important cause of thunderclap headache, commonly reversible, but several neurological complications including seizure, ischemic infarcts, and hemorrhage may happen (4)

Notes:

• posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are relatively uncommon neurological disorders, but their detection has been increasing mainly due to clinical awareness and spreading of magnetic resonance imaging (MRI) (4)
  • these syndromes share some common clinical and radiologic features and occasionally occur in the same patient, misdiagnosis may occur
  • PRES
    • is characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficit, confusion, disorders of consciousness, seizures, and motor deficits often associated to peculiar neuroradiological pattern even if uncommon localization and ischemic or hemorrhagic lesions were described
    • risk factors of PRES include:
      • immunosuppression,
      • malignancy,
      • pre-eclampsia,
      • renal failure,
      • autoimmune disorders,
      • sepsis, hypertension,
      • transplantation, and
      • chemotherapeutic medications
      • may occur also in healthy subjects
  • RCVS
    • is a group of diseases typically associated with severe headaches and reversible segmental vasoconstriction of cerebral arteries, often complicated by ischemic or hemorrhagic stroke

Reference:

• (1) Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906-17
• (2) Sattar A, Manousakis G, Jensen MB. Systemic review of reversible cerebral vasoconstriction syndrome.  Expert Rev Cardiovasc Ther. 2010; 8(10): 1417-1421
• (3) Ducros A, Bousser MG. Thunderclap headache. BMJ. 2013;346:e8557
• (4) Pilato F, Distefano M, Calandrelli R. Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome: Clinical and Radiological Considerations. Front Neurol. 2020 Feb 14;11:34. doi: 10.3389/fneur.2020.00034. PMID: 32117007; PMCID: PMC7033494.