Rare association of posterior reversible encephalopathy syndrome (PRES) with hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome - A case report and review of the literature

Abstract

Introduction: The hemolysis-elevated liver enzymes-low platelet counts (HELLP) syndrome and posterior reversible encephalopathy syndrome (PRES) are rare conditions that can complicate pregnancy and the early postpartum period. Although both are closely related to hypertensive pregnancy disorders, their association is rarely described, so the outcome of these patients remains unknown. We present a case report of PRES associated with HELLP syndrome and a review of all previously published cases, including demographic characteristics, clinical presentation, treatment, and outcome.

Case presentation: A previously healthy 31-year-old woman in the 38th week of pregnancy was admitted to the obstetric department due to elevated blood pressure. The first laboratory findings were consistent with HELLP, which is why she was delivered by emergency caesarean section. Forty-four hours after the cesarean section, she presented with a severe headache, blurred vision, and instability, followed by two seizures. Magnetic resonance imaging (MRI) of the brain showed T2-weighted/FLAIR left-sided hyperintensity consistent with PRES. She was treated with antihypertensive, antiedematous, and other symptomatic therapy. The control brain MRI showed complete regression of the previously described changes, and she was discharged without a neurological deficit. So far, 33 cases of HELLP associated with PRES have been reported in the literature, including our case. Our review of 30 cases showed that although most patients have good outcomes, if not treated in time, patients may develop serious somatic complications and permanent neurological deficits, or even fatal outcomes.

Conclusion: PRES associated with HELLP syndrome is a rare combination of third trimester/postpartum complications which usually present with seizures, altered sensorium, headache, hypertension, and laboratory disorders. These disorders require mutual neurological, internal and gynaecological treatment, and if timely treated resolve completely in most of the cases. However, caution is required as these patients may develop serious somatic complications and permanent neurological deficits or even fatal outcomes.

Keywords: Case report; HELLP; Outcome; PRES; Pregnancy.

Fig. 1

a) Initial brain MRI/T2-weighted transversal plane section (3rd day postpartum)-hyperintensity in the left occipital lobe; b) Initial brain MRI/T2-weighted sagittal plane section (3rd day postpartum)- hyperintensity in the left occipital lobe; c) Initial brain MRI/FLAIR axial plane section (3rd day postpartum)- hyperintensity in the left occipital lobe; d) Control brain MRI/T2-weighted transversal plane section (17th day postpartum)- normal finding; e) Control brain MRI/T2-weighted sagittal plane section (17th day postpartum)- normal finding; f) Control brain MRI/FLAIR axial plane section (17th day postpartum)- normal finding. MRI- magnetic resonance imaging; FLAIR – fluid attenuated inversion recovery.

Graph 1

Time course of the patient's illness, including clinical picture, diagnostic results, prescribed therapy and outcome. IVF = in vitro fertilization; BP = blood pressure; HELLP= Hemolysis, Elevated Liver enzymes and Low Platelets; NICU = neurology intensive care unit; CT= Computed Tomography; MRI = Magnetic Resonance Imaging; PRES= Posterior Reversible Encephalopathy Syndrome; TCD = Transcranial doppler.