Baylor University Medical Center Proceedings April 2017 - 220

<100 mg/dL, <15,000 white blood cells/μL); 4) severe, acute
headaches, with or without additional neurologic signs or symptoms; and 5) reversibility of angiographic abnormalities within
12 weeks of onset.
RCVS may be primary (idiopathic) or secondary (60%
to 80%) with multiple precipitants (5). The most common
are vasoactive substance use and a postpartum state. The exact
pathogenesis of RCVS is unknown, with "disturbance of cerebral vascular tone" being the most widely accepted hypothesis.
Primary angiitis of the central nervous system (PACNS)
was recognized to be distinct from RCVS only in 1990 (3).
Headaches tend to be more insidious in PACNS, which follows
a more progressive-chronic course with a stepwise deterioration
(3, 5). CSF is significant for a marked inflammatory profile and
can also reveal oligoclonal bands. Brain magnetic resonance
imaging may reveal deep infarcts and contrast enhancements
of the vessel wall (3, 4). It is of some importance to accurately
distinguish these two entities, since treatment for PACNS with

220

corticosteroids can be harmful in RCVS. Conversely, RCVS
stabilizes and improves more quickly (3). There is no specific
proven therapy for RCVS.
1.

2.
3.
4.
5.
6.

7.

Topcuoglu MA, Chan ST, Silva GS, Smith EE, Kwong KK, Singhal AB.
Cerebral vasomotor reactivity in reversible cerebral vasoconstriction syndrome. Cephalalgia 2016 May 20 [Epub ahead of print].
Sorensen DM. Reversible cerebral vasoconstriction syndrome. JAMA
Neurol 2016;73(2):232-233.
Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol
2012;11(10):906-917.
Mehdi A, Hajj-Ali RA. Reversible cerebral vasoconstriction syndrome: a
comprehensive update. Curr Pain Headache Rep 2014;18(9):443.
Bernard KRL, Rivera M. Reversible cerebral vasoconstriction syndrome.
Selected topics: neurological emergencies. J Emerg Med 2015;49(1):26-31.
Kobayashi M. Diffuse cerebrovascular dilation: case report of amezinium
metilsulfate-induced reversible cerebral vasoconstriction syndrome. Cephalalgia 2016;36(3):289-293.
Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med
2007;146(1):34-44.

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