Cortical blindness in severe preeclampsia: computed tomography, magnetic resonance imaging, and single-photon-emission computed tomography findings

doi:10.1016/S0029-7844(00)00878-4

Obstetrics & Gynecology

Volume 95, Issue 6, Part 2, June 2000, Pages 1017-1019

https://doi.org/10.1016/S0029-7844(00)00878-4 Get rights and content

Abstract

Background: Cortical blindness is a complication of severe preeclampsia, but it is unclear whether it results from cerebral vasospasm and ischemic injury or vasogenic (hydrostatic) edema due to increased capillary permeability.

Case: Reversible cortical blindness in a 33-year-old gravida 2, para 1, with severe postpartum preeclampsia after evacuation of a partial molar pregnancy at 19 weeks’ gestation is presented. Initial neuroimaging studies showed hyperperfusion on head single-photon-emission computed tomography scan, which corresponded with lesions found on head computed tomography and magnetic resonance imaging scans. Follow-up neuroimaging studies 2 weeks later, by which time the patient’s visual acuity had returned to normal, showed complete resolution of radiologic abnormalities.

Conclusion: Neuroimaging studies in a woman with severe postpartum preeclampsia complicated by reversible cortical blindness showed that blindness resulted from vasogenic (hydrostatic) cerebral edema and not cerebral vasospasm.

Section snippets

Case

A 33-year-old woman, gravida 2, para 1, had abdominal ultrasound at 18 weeks’ gestation that found a probable partial molar pregnancy with a single structurally anomalous female fetus. Antenatal course was complicated by intractable nausea and vomiting. Results of pertinent laboratory tests were β-hCG 1,263,520 mIU/mL and TSH 0.44 mU/L (normal range, 0.2–0.40 mU/L). An uncomplicated dilation and evacuation was done with an autopsy that confirmed the multiple fetal anomalies detected by

Comment

The incidence of cortical blindness manifestated by hypertensive encephalopathy in preeclampsia is 1–15%.5, 7 It is not known whether cortical blindness results from cerebral vasospasm with ischemic injury, arteriolar necrosis, and cytotoxic edema3, 4 or from increased capillary permeability with leakage of fluid and proteins into the surrounding tissues resulting in vasogenic (hydrostatic) edema.5, 6 In either case, the end result is focal cerebral edema. The cerebral edema on head CT or MRI

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Transient cortical blindness in preeclampsia with indication of generalized vascular endothelial damage
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Cited by (77)

Optical coherence tomography angiography assessment of retinochoroidal microcirculation differences in preeclampsia
2024, Photodiagnosis and Photodynamic Therapy
To investigate microvascular changes in pregnant women with preeclampsia using optical coherence tomography angiography (OCTA) and compare the results with healthy pregnant and non-pregnant subjects.
Superficial capillary plexus (SCP), deep capillary plexus (DCP) choriocapillaris (CC) vessel density (VD) and foveal avascular zone area (FAZ), retina, retinal nerve fiber layer (RNFL), the ganglion cell layer (GCL) and the choroidal thickness were examined and compared in preeclamptic pregnant (group 1), healthy pregnant women (group 2) and non-pregnant, age-matched female controls (group 3). The correlations of the parameters with each other and with blood pressure were evaluated.
No significant difference was found between the groups when retinal, RNFL and GCL thickness values (p> 0.05). The choroidal thickness values were significantly lower in group 1 than in group 2 (p = 0.029). The central foveal VD of the SCP and DCP was significantly lower in group 1 compared to groups 2 and 3 (p = 0.03, p< 0.01 respectively). The mean VD of the SCP was significantly higher in groups 1 and 2 than in group 3 (p = 0.01). The FAZ area was statistically significantly lower in group 3 than in group 2 (p = 0.032). The CC VD was lower in group 3 compared to the other groups in all measurements (p < 0.01).The FAZ area was positively correlated with systolic blood pressure in group 1.
The use of OCTA, a non-invasive imaging technique, to assess the retinal microcirculation appears to have the potential to in the early diagnosis or follow up in preeclampsia before signs of hypertensive retinopathy.
Cerebral perfusion in posterior reversible encephalopathy syndrome measured with arterial spin labeling MRI
2022, NeuroImage: Clinical
Citation Excerpt :
With this theory, vasoconstrictive and immunogenic agents released by damaged vascular endothelial cells are thought to mediate vasospasm and/or increased BBB permeability, leading to cerebral hypoperfusion and edema formation (Bartynski, 2008; Anderson et al., 2020; Marra et al., 2014). Prior studies using contrast-enhanced MR and CT perfusion imaging and Tc99m-hexamethylpropyleneamine oxime (Tc99m-HMPAO) SPECT have yielded contradictory results (Bartynski, 2008; Schwartz et al., 1995; Apollon et al., 2000; Hedna et al., 2012; Wartenberg and Parra, 2006; Vanacker et al., 2015; Sarbu et al., 2014). While a number of early case reports and series have reported hyperperfusion in the setting of PRES (Schwartz et al., 1995; Apollon et al., 2000; Hedna et al., 2012; Wartenberg and Parra, 2006), more recent studies have shown reduced CBF in affected regions (Vanacker et al., 2015; Sarbu et al., 2014).
The pathophysiologic basis of posterior reversible encephalopathy syndrome (PRES) remains controversial. Hypertension (HTN)-induced autoregulatory failure with subsequent hyperperfusion is the leading hypothesis, whereas alternative theories suggest vasoconstriction-induced hypoperfusion as the underlying mechanism. Studies using contrast-based CT and MR perfusion imaging have yielded contradictory results supporting both ideas. This work represents one of the first applications of arterial spin labeling (ASL) to evaluate cerebral blood flow (CBF) changes in PRES.
After obtaining Institutional Review Board approval, MRI reports at our institution from 07/2015 to 09/2020 were retrospectively searched and reviewed for mention of “PRES” and “posterior reversible encephalopathy syndrome.” Of the resulting 103 MRIs (performed on GE 1.5 Tesla or 3 Tesla scanners), 20 MRIs in 18 patients who met the inclusion criteria of clinical and imaging diagnosis of PRES and had diagnostic-quality pseudocontinuous ASL scans were included. Patients with a more likely alternative diagnosis, technically non-diagnostic ASL, or other intracranial abnormalities limiting assessment of underlying PRES features were excluded. Perfusion in FLAIR-affected brain regions was qualitatively assessed using ASL and characterized as hyperperfusion, normal, or hypoperfusion. Additional quantitative analysis was performed by measuring average gray matter CBF in abnormal versus normal brain regions.
HTN was the most common PRES etiology (65%). ASL showed hyperperfusion in 13 cases and normal perfusion in 7 cases. A hypoperfusion pattern was not identified. Quantitative analysis of gray matter CBF among patients with visually apparent hyperperfusion showed statistically higher perfusion in affected versus normal appearing brain regions (median CBF 100.4 ml/100 g-min vs. 61.0 ml/ 100 g-min, p < 0.001).
Elevated ASL CBF was seen in the majority (65%) of patients with PRES, favoring the autoregulatory failure hypothesis as a predominant mechanism. Our data support ASL as a practical way to assess and noninvasively monitor cerebral perfusion in PRES that could potentially alter management strategies.
Cerebrovascular Pathophysiology in Preeclampsia and Eclampsia
2021, Chesley’s Hypertensive Disorders in Pregnancy
Hemorrhagic and ischemic strokes are, in addition to eclamptic fits, among the most important and feared causes of maternal morbidity and mortality in women with preeclampsia. Increasing evidence now indicates that both preeclampsia and eclampsia can impact long-term maternal cerebrovascular health. This chapter includes updated clinical, epidemiological, and mechanistic knowledge since the last edition, and adds important new sections on “Stroke in Pregnancy and Preeclampsia” and “Neurocognitive Functioning.” Future basic, translational, and clinical research are urgently needed to improve our mechanistic understanding of how preeclampsia and eclampsia damage the maternal brain, hopefully leading to improved prevention and treatment strategies. The important contributions of previous authors of this chapter, Gerda G. Zeeman and F. Gary Cunningham, are greatly appreciated.
Immune system activation in the pathogenesis of posterior reversible encephalopathy syndrome
2017, Brain Research Bulletin
Citation Excerpt :
Direct evidence of cerebral hyperperfusion is not usually found in PRES patients. Hyperperfusion was only reported in some of the early PRES cases (Schwartz et al., 1992; Apollon et al., 2000). Most of the PRES patients reported previously had high blood pressure, especially patients with eclampsia and hypertension.
Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome characterized by a variable combination of headaches, seizures, altered mental status, visual impairment, focal neurological signs and symmetric vasogenic edema in bilateral posterior cerebral circulation territory. The pathogenesis of PRES is still controversial. Most of the clinical conditions associated with PRES involve a systemic toxicity response in the entire organism with activation of the cells of the immune system and cytokines. These PRES related conditions induce T cell activation, cytokine release, and subsequent leukocyte adhesion and activation, resulting in endothelial damage and fluid leakage. This potential mechanism of immune system activation and endothelial dysfunction may play a critical role in the pathogenesis of PRES. In this review, the role of immune system activation and endothelial dysfunction in the pathogenesis of PRES is discussed, with the aim to improve our understanding of this disorder.
Neuroimaging features in posterior reversible encephalopathy syndrome: A pictorial review
2017, Journal of the Neurological Sciences
Posterior reversible encephalopathy syndrome (PRES) is a radioclinical entity associating nonspecific neurological symptoms (headache, seizures, impairment of alertness, visual disturbances…) occurring in evocative clinical condition (hypertension, eclampsia, immunosuppressor agents, systemic lupus erythematosus…). In the acute stage, the typical imaging finding is a vasogenic edema predominant in the subcortical parietal-occipital white matter.
The purpose of this pictorial review is to illustrate the different neuroimaging features of PRES and present key radiological elements to assert diagnosis. In this overview, we examine the following points: the distributions of vasogenic edema, hemorrhage, the varying patterns in diffusion and perfusion, the different types of enhancement encountered and the vascular modifications demonstrated by angiography. The cause of PRES is still unknown. Nevertheless, catheter angiography, MR angiography and MR perfusion features in PRES render further insight into its pathophysiology.
Follow-up imaging shows evidence of radiologic improvement in the very large majority of cases in 1 or 2 weeks, sometimes in up to 1 month. Recurrent PRES attacks are uncommon.
Atypical imaging presentation should not reject the diagnosis of PRES in a compatible clinical situation.
Posterior reversible encephalopathy in the intensive care unit
2017, Handbook of Clinical Neurology
Posterior reversible encephalopathy syndrome (PRES) is increasingly diagnosed in the emergency department, and medical and surgical intensive care units. PRES is characterized by acute onset of neurologic symptoms in the setting of blood pressure fluctuations, eclampsia, autoimmune disease, transplantation, renal failure, or exposure to immunosuppressive or cytotoxic drugs, triggers known to admit patients to the intensive care unit (ICU). Although the exact pathophysiology remains unknown, there is growing consensus that PRES results from endothelial dysfunction. Because of the heterogeneous nature of the disorder, it is probable that different mechanisms of endothelial injury are etiologically important in different clinical situations. The presence of bilateral vasogenic edema on brain imaging, particularly in parieto-occipital regions, is of great diagnostic utility but PRES remains a clinical diagnosis. Although largely reversible, PRES can result in irreversible neurologic injury and even death. The range of clinical and radiographic manifestations of the syndrome is probably broader than previously thought, and it is imperative that clinicians become familiar with the full spectrum of the disorder, as prompt recognition and elimination of an inciting factor improve outcome. PRES may be the most frequent toxic-metabolic encephalopathy seen in the ICU.

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^☆: Supported by the Reproductive Scientist Development Program through National Institutes of Health (grant 5K12-HD00849) and the Association of Professors of Gynecology and Obstetrics (to ERN).

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ObstetricsCortical blindness in severe preeclampsia: computed tomography, magnetic resonance imaging, and single-photon-emission computed tomography findings☆

Abstract

Section snippets

Case

Comment

Am J Ophthalmol

Am J Obstet Gynecol

Transient cortical blindness in preeclampsia with indication of generalized vascular endothelial damage

J Neuroophthalmol

Obstetrics
Cortical blindness in severe preeclampsia: computed tomography, magnetic resonance imaging, and single-photon-emission computed tomography findings☆