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Call-Fleming Syndrome (RCVS)
MRA of Head. Demonstrates diffuse segmental areas of vasoconstriction (image on the right) and subsequent normal filling in the same patient 3 months later (image on the left).

Call-Fleming Syndrome (RCVS)

Overview
  • Reversible cerebral vasoconstriction syndrome (RCVS) (aka Call-Fleming Syndrome) is a disorder of diffuse segmental cerebral vasoconstriction that spontaneously resolves within 3 months.
Clinical Manifestations
Primary manifestations include:
  • Thunderclap headache (typically severe for a couple of hours (typically shorter than from aneurysmal rupture). Patients typically suffer 4 attacks during the course of the illness with persistent dull headache in between. Headache triggers: exertion/valsalva/emotional stress
  • Stroke: Ischemic and/or Hemorrhagic, causing focal deficits or seizures.
Causes
Common Triggers:
  • Post-partum (known as: Post-partum angiopathy)
  • Vasoactive pharmaceuticals
    • Illicit drugs
    • SSRIs/SNRIs
    • Triptans
    • Nicotine
    • Hormonal therapies (oral contraceptives, hormone replacement therapies): initiation or discontinuation can precipitate RCVS.
  • Pheochromocytoma (Catecholamine Producing Tumors)
Important other causes of thunderclap headache:
  • Subarachnoid hemorrhage
  • Obstructive hydrocephalus (eg, 3rd ventricle colloid cyst tumor)
  • Acute sinusitis
  • Meningitis
  • Dural venous sinus thrombosis
  • Carotid or vertebral artery dissection
  • Pituitary apoplexy
  • Intracranial hypotension (CSF pressure low)
Radiographic Findings
  • "Sausage on a string appearance": Alternating tapered narrowing and abnormal dilation of 2nd and 3rd order cerebral arteries (eg, the M2 and M3 branches of the MCA).
  • Nonaneurysmal cortical subarachnoid hemorrhage.
  • Wedge shaped infarctions (note that venous infarcts characteristically have a wedge shape infarct, as well).
  • Cerebral edema.
Overlap Syndromes