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Original Contributions |

Fulminant Postpartum Cerebral Vasoconstriction Syndrome

Jennifer E. Fugate, DO; Eelco F. M. Wijdicks, MD, PhD; Joseph E. Parisi, MD; David F. Kallmes, MD; Harry J. Cloft, MD, PhD; Kelly D. Flemming, MD; Elias A. Giraldo, MD, MS; Alejandro A. Rabinstein, MD
Arch Neurol. 2012;69(1):111-117. doi:10.1001/archneurol.2011.811.
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Objective To raise awareness of the potentially adverse consequences of postpartum cerebral vasoconstriction, which is typically considered benign and self-limiting, by describing 4 fulminantly fatal cases.

Design Retrospective case series.

Setting Tertiary referral center.

Patients Four postpartum women aged 15 to 33 years developed acute neurologic deficits 1 to 8 days after uncomplicated deliveries. One had a history of migraine headaches and 2 had histories of spontaneous abortion. Two of the patients had uneventful pregnancies and 2 had preeclampsia, 1 of whom had acute hepatic failure. Presenting symptoms included severe headache (n = 3), focal deficit (n = 1), seizure (n = 1), and encephalopathy (n = 1). Initial brain imaging results demonstrated cortical ischemia and global edema in 2 patients, lobar hemorrhage in 1, and normal findings in 1. All had rapid clinical deterioration from hours to days with multiterritorial infarctions and global brain edema on imaging. All had angiographic findings of diffuse, severe, segmental multifocal arterial narrowings.

Interventions Aggressive treatment was attempted with most patients including intravenous magnesium sulfate, corticosteroids, calcium channel blockers, balloon angioplasty, vasopressors, and osmotic agents. Two patients underwent serial angiography, with results showing severe, recurrent proximal vasoconstriction involving all major intracranial vessels.

Results All patients had fulminant, accelerating courses leading to their deaths within 8 to 24 days after delivery.

Conclusions Postpartum vasoconstriction can be fatal, with rapid progression of vasoconstriction, ischemia, and brain edema. Clinicians need to be aware of the potential consequences of this condition. Postpartum women with acute neurologic symptoms require prompt investigation with noninvasive cerebrovascular imaging and close monitoring for possible secondary deterioration.

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Figure 1. Imaging findings in cases 1 and 2 with fulminant postpartum vasoconstriction syndrome. A, Axial fluid-attenuated inversion recovery magnetic resonance imaging in case 1 shows areas of T2 hyperintensity corresponding with acute ischemia. B, Catheter angiogram shows severe stenosis of the distal right internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery (arrows) with poor anterograde flow despite a delayed arterial phase. C, Noncontrast head computed tomographic scan shows subacute left basal ganglia hemorrhage. D, In case 2, noncontrast head computed tomographic scan shows diffuse cerebral edema with cisternal effacement. E, Angiogram (left vertebral artery injection) shows marked vasculopathy with beads on string appearance in the basilar artery, its branches, and both posterior cerebral arteries (arrows). F, A noncontrast head computed tomographic scan early in the course demonstrates acute right intraparenchymal hemorrhage in the basal ganglia (arrow).

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Figure 2. Photomicrographs from postmortem examination in case 1. A, Movat pentachrome staining reveals normal structures of the arterial wall in a section of the M1 segment of the right middle cerebral artery (original magnification ×200). B, Movat pentachrome staining shows subtle focal attenuation and thinning of the media of uncertain significance (arrow) in a section of the A1 segment of the right anterior cerebral artery (original magnification ×200).

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Figure 3. Imaging findings in cases 3 and 4 with fulminant postpartum vasoconstriction syndrome. A, Axial diffusion-weighted magnetic resonance imaging in case 3 shows cortical restricted diffusion. B, Magnetic resonance angiograph shows diffuse arterial irregularities. C, Sulcal and basilar cistern effacement on a head computed tomographic scan in case 4 shows global edema. D, Computed tomographic angiogram in case 4 shows severe diffuse narrowing of intracranial arteries, which have a beaded appearance.

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Figure 4. Photomicrographs from postmortem examination in case 4. Section of the M1 segment of the left middle cerebral artery shows mild focal intimal hyperplasia (arrows) on staining with hematoxline-eosin (original magnification ×100) (A) and Movat pentachrome (original magnification ×100) (B).

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