(P91) The Posterior Reversible Encephalopathy Syndrome (PRES) at the ED - A Case Study

Chief Complaint : A 29-year-old female patient was admitted in the Emergency Department of Semmelweis University (Hungary) with repeated seizures after having had headache and hypertension in the past few days. She had a C-section 6 days prior.

History of Present Illness : On arrival at the clinic, the patient had a repeated convulsion. After physical examination and stabilization, laboratory tests and urgent non-contrast cranial CT were performed and symptomatic therapy was continued. Imaging ruled out intracranial bleeding, but described radiological features of PRES. To confirm the diagnosis, an urgent contrast-mediated head MRI was performed where the corresponding perfusion sequences demonstrated radiomorphological sign of a PRES. As the radiological findings were in line with the clinical picture, the diagnosis of post-partum eclampsia and subsequent PRES was made.
Further, multidisciplinary care was performed with the participation of the Department of Neurology, Obstetrics and Gynaecology, and Anaesthesiology and Intensive Care of Semmelweis University. The EEG examination did not confirm active seizure activity, but due to cumulative seizures antiepileptic therapy (levetiracetam) was initiated. Treatment of hypertension was continued with intravenous urapidil therapy. A transvaginal ultrasound showed coagulum, during the curettage a large amount of coagulum was removed from the uterus. Subsequently, the patient's blood pressure rapidly decreased and she was switched to per os medication.
On further observation of the patient, her complaints resolved. Follow-up MRI 14 days after initial cranial MRI showed significant regression.

Pertinent Physical Exam: The patient was somnolent (GCS of 3-5-6) with left sided (upper and lower limb) hemiparesis and hyperreflexia. airway and breathing assessment was negative but severe hypertension (210/145) was noted during physical examination. Initial blood gas analysis showed marked lactic acidosis (pH 7,29, Lac 7,2) and euglycemia (Glu 6,5 mmol/l). In the ED the patient was initially managed with intravenous (IV) magnesium (5g), IV benzodiazepine (clonazepam 0,5 mg), phenytoin (250 mg bolus and 750 mg/10 hours in perfusor), and received labetalol (3x10mg). During the ED stay, her blood pressure was adequately controlled, and no further convulsive activity was observed.
Due to the PRES confirmed by radiological imaging, the patient was further managed with the involvement of several co-clinicians.

Pertinent Laboratory Data: Laboratory studies revealed elevated CRP and reduced renal function, however there was no evidence of liver, kidney or other potential organ dysfunction or HELLP syndrome. The elevation of inflammatory markers was due to postpartum, postoperative period. Thus, in agreement with the radiological findings, the clinical diagnosis was PRES syndrome due to postpartum eclampsia.

Case Discussion: The posterior reversible encephalopathy syndrome (PRES) is a rare, severe, but potentially reversible clinical and neuroradiologycal disorder. The diverse clinical symptoms can occur without any direct link to age-group or medical history. Early recognition, diagnostics, and treatment are key elements to reverse encephalopathy syndrome.
Pathology: the exact mechanism of PRES is not known, but it is thought to be related to endothelial cell dysfunction and altered blood-brain barrier integrity, with consequent cortical and subcortical vasogenic oedema.
Etiology: many diseases should be considered in association with the possibility of PRES, the most common conditions are untreated hypertension, eclampsia, sepsis, autoimmune disease (TTP, SLE), COVID infection, drug toxicity (cisplatin, interferon, erythropoietin, cyclosporin), bone marrow or stem cell transplantation, organ transplantation, sepsis, COVID-19 infection, HELLP syndrome.
Clinical manifestations: clinical signs and symptoms of PRES are often non-specific and take hours or days to develop. Red flag symptoms are characterized by headache, visual disturbance, altered mental state, seizures, vomiting and cortical blindness.
Radiological presentation: clinical suspicion of PRES can be confirmed by cranial CT or magnetic resonance imaging. The most characteristic visual appearance is the presence of reversible vasogenic subcortical oedema affecting the white matter of the posterior part of both hemispheres. The frontal, temporal and cerebellar lobes are also frequently affected.
Treatment:
PRES is still a reversible condition if adequate treatment is started within a few days. If treatment is inadequate or delayed, there is a high risk of irreversible neurological damage, cerebral infarction or haemorrhage, even death.
Treatment includes management of the primary lesion, as well as control of neurological symptoms and hypertension, with emphasis on antihypertensive agents (e.g. labetalol, hydralazine, nicardipine, nifedipine), initiation of hydration with crystalloid solutions, ensuring adequate oxygenation, treatment of seizure activity (magnesium sulphate; diazepam, phenytoin for epilepsy). Diuretics, corticosteroids (e.g. dexamethasone, bethamethasone) are complementary therapeutic options.

References and Acknowledgements (Optional): Tetsuka S, Ogawa T. Posterior reversible encephalopathy syndrome: A review with emphasis on neuroimaging characteristics. (2019) Journal of the neurological sciences. 404: 72-79.