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E-043 Reversible Cerebral Vasoconstriction Syndrome: Non-invasive Imaging Findings and Angiographic Evaluation
  1. K de Macedo Rodrigues,
  2. R Hito,
  3. D Takhtani,
  4. J Lozano,
  5. A Wakhloo,
  6. A Puri
  1. Radiology, University of Massachusetts Medical School, Worcester, MA

Abstract

Purpose Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a transient disorder of cerebral arterial auto regulation that may have a non-specific clinical presentation, most typically initiating as an acute episode of severe (thunderclap) headache. The clinical scenario and the non-invasive imaging findings are similar to those seen in more common pathologies making it a challenging diagnosis if the entity is not a consideration in the initial evaluation. RCVS presentation and initial imaging findings may mimic a ruptured aneurysm, stroke, migraine and posterior reversible encephalopathy syndrome, among others. Our aim is to describe the entity of RCVS and demonstrate the common and uncommon imaging findings seen on CT, MRI, CT angiography, with emphasis on conventional digital subtraction arteriogram pre and post endovascular treatment.

Approach/methods A multimodality pictorial review of cases from our institution is presented, including non-contrast head CT, CT angiogram, MRI, MR angiogram and conventional digital subtraction arteriogram (DSA). Diagnosis of RCVS was confirmed by imaging demonstration of reversibility of arterial constriction on follow-up imaging or after intra-arterial therapy.

Findings/discussion We present indirect findings on non-invasive imaging, such as transient cerebral edema, convexal subarachnoid hemorrhage, intraparenchymal hemorrhage and cerebral. Direct signs of single or multivessel arterial vasoconstriction are seen on CTA, MRA and confirmed on DSA. Reversibility of the vasoconstriction is demonstrated after intra-arterial administration of calcium channel blocker. Conditions such as diffuse atherosclerotic arterial narrowing, vasculitis, posterior reversible encephalopathy, cerebral edema secondary to venous congestion and vasospasm secondary to aneurysmal subarachnoid hemorrhage were among the pathologies considered prior to a definitive diagnosis of RCVS.

Summary/conclusion Familiarity with the imaging findings of RCVS is of critical importance to direct appropriate and timely clinical management. Atypical subarachnoid hemorrhage, unexplained cerebral edema and hemorrhage, as well as single or multivessel diffuse narrowing with a relatively normal appearing brain should raise suspicion of RCVS. Suspicion can be confirmed by demonstrating the reversibility of vasoconstrictions after intra-arterial administration of calcium channel blocker or at follow-up imaging.

Abstract E-043 Figure 1

48-year old female presenting with severe headache and evidence of subarachnoid hemorrhage on head CT (not shown). No aneurysm or other causes for intracranial bleed was demonstrated on DSA. (A) Multiple segmentel areas of vasoconstrictions of the MCA and ACA branches are demonstrated. (B) There is significant improvement of the degree if vasoconstriction after intra-arterial administration of calcium channel blocker.

Disclosures K. de Macedo Rodrigues: None. R. Hito: None. D. Takhtani: None. J. Lozano: None. A. Wakhloo: 1; C; NIH, Philips Healthcare, Wyss Institute. 2; C; Codman Neurovascular and Stryker Neurovascular. 4; C; co-founder of InNeuroCo Inc. and major stockholder; stocks in EpiEB and Pulsar Medical. 6; C; speaker: Harvard Postgraduate Course, Miami Cardiovascular Institute. A. Puri: 1; C; Stryker Neurovascular and Covidien. 2; C; Codman Neurovascular, Stryker Neurovascular and Covidien. 4; C; InNeuroCo Inc. 6; C; speaker: Miami Cardiovascular Institute.

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work noncommercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

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