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).
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).
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.
"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).