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).
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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
- Note that there is an important pathophysiological overlap with posterior reversible leukoencephalopathy (PRES).
- Also note that ~ 10% with RCVS will have concomitant cervical artery dissections (carotid or vertebral artery dissections).