Fulminant Postpartum Cerebral Vasoconstriction Syndrome

Abstract

To raise awareness of the potentially adverse consequences of postpartum cerebral vasoconstriction, which is typically considered benign and selflimiting, 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.

Association between preeclampsia and reversible cerebral vasoconstriction syndrome: A case‐control study

“…Prior studies of pregnancy‐associated RCVS are limited to case series and have not quantified the risk of RCVS related to preeclampsia‐eclampsia. One retrospective analysis of “postpartum angiopathy” (an earlier term for RCVS) identified 18 patients, among which 39% had preeclampsia‐eclampsia, compared to a much higher prevalence of 72% in our cases [15]. However, patients in that series had poorer outcomes with a 50% rate of residual deficits or mortality, compared to a 15% need for rehabilitation at discharge and no mortality seen in our series.…”

Association between preeclampsia and reversible cerebral vasoconstriction syndrome: A case‐control study

“…Prior studies of pregnancy‐associated RCVS are limited to case series and have not quantified the risk of RCVS related to preeclampsia‐eclampsia. One retrospective analysis of “postpartum angiopathy” (an earlier term for RCVS) identified 18 patients, among which 39% had preeclampsia‐eclampsia, compared to a much higher prevalence of 72% in our cases [15]. However, patients in that series had poorer outcomes with a 50% rate of residual deficits or mortality, compared to a 15% need for rehabilitation at discharge and no mortality seen in our series.…”

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

“…Prior studies have shown that 16%-34% of patients develop transient neurologic deficits, including seizures, but fewer than 10% develop mild to moderate permanent deficits and only a minority show relentless progression that can be fatal. 2,6,10,19,26 Ideally, specific treatments should be targeted to patients with high likelihood for poor outcome. At present it is challenging to identify such patients and intervene before significant irreversible damage occurs.…”

Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

“…We found 3 isolated case‐reports with RCVS without headache . One young woman with postpartum RCVS presented with coma .…”

“…19 Impaired consciousness may have precluded the description of a headache in this patient. 19 The 2 other patients presented with acute focal deficits revealing an IS, which was attributed to RCVS after thorough investigations and serial cerebral angiographies. 23,30 The absence of headache in these 2 case-reports confirms the results of the French series of IS revealing an RCVS, 17 showing that RCVS can cause IS without any preceding or associated headache.…”

Association between preeclampsia and reversible cerebral vasoconstriction syndrome: A case‐control study

“…Prior studies of pregnancy‐associated RCVS are limited to case series and have not quantified the risk of RCVS related to preeclampsia‐eclampsia. One retrospective analysis of “postpartum angiopathy” (an earlier term for RCVS) identified 18 patients, among which 39% had preeclampsia‐eclampsia, compared to a much higher prevalence of 72% in our cases [15]. However, patients in that series had poorer outcomes with a 50% rate of residual deficits or mortality, compared to a 15% need for rehabilitation at discharge and no mortality seen in our series.…”

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

“…Prior studies have shown that 16%-34% of patients develop transient neurologic deficits, including seizures, but fewer than 10% develop mild to moderate permanent deficits and only a minority show relentless progression that can be fatal. 2,6,10,19,26 Ideally, specific treatments should be targeted to patients with high likelihood for poor outcome. At present it is challenging to identify such patients and intervene before significant irreversible damage occurs.…”

Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

“…We found 3 isolated case‐reports with RCVS without headache . One young woman with postpartum RCVS presented with coma .…”

“…19 Impaired consciousness may have precluded the description of a headache in this patient. 19 The 2 other patients presented with acute focal deficits revealing an IS, which was attributed to RCVS after thorough investigations and serial cerebral angiographies. 23,30 The absence of headache in these 2 case-reports confirms the results of the French series of IS revealing an RCVS, 17 showing that RCVS can cause IS without any preceding or associated headache.…”

Association between preeclampsia and reversible cerebral vasoconstriction syndrome: A case‐control study

“…Prior studies of pregnancy‐associated RCVS are limited to case series and have not quantified the risk of RCVS related to preeclampsia‐eclampsia. One retrospective analysis of “postpartum angiopathy” (an earlier term for RCVS) identified 18 patients, among which 39% had preeclampsia‐eclampsia, compared to a much higher prevalence of 72% in our cases [15]. However, patients in that series had poorer outcomes with a 50% rate of residual deficits or mortality, compared to a 15% need for rehabilitation at discharge and no mortality seen in our series.…”

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

“…Prior studies have shown that 16%-34% of patients develop transient neurologic deficits, including seizures, but fewer than 10% develop mild to moderate permanent deficits and only a minority show relentless progression that can be fatal. 2,6,10,19,26 Ideally, specific treatments should be targeted to patients with high likelihood for poor outcome. At present it is challenging to identify such patients and intervene before significant irreversible damage occurs.…”

Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

“…We found 3 isolated case‐reports with RCVS without headache . One young woman with postpartum RCVS presented with coma .…”

“…19 Impaired consciousness may have precluded the description of a headache in this patient. 19 The 2 other patients presented with acute focal deficits revealing an IS, which was attributed to RCVS after thorough investigations and serial cerebral angiographies. 23,30 The absence of headache in these 2 case-reports confirms the results of the French series of IS revealing an RCVS, 17 showing that RCVS can cause IS without any preceding or associated headache.…”