Posterior reversible encephalopathy syndrome: A case report on visual outcome in a patient with pre-eclampsia

Nishi Prasad; Aditi Dubey; Kavita Kumar

doi:10.4103/IJO.IJO_3307_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 475-477

Posterior reversible encephalopathy syndrome: A case report on visual outcome in a patient with pre-eclampsia

Nishi Prasad, Aditi Dubey, Kavita Kumar
Department of Ophthalmology, Gandhi Medical College, Bhopal, Madhya Pradesh, India

Date of Submission	20-Dec-2022
Date of Acceptance	08-Feb-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Nishi Prasad
Room-118, H Block PG Girls Hostel, GMC Campus, Sultania Road, Bhopal - 462001, Madhya Pradesh
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_3307_22

Abstract

Posterior reversible encephalopathy syndrome (PRES), also known as reversible posterior leukoencephalopathy syndrome or reversible posterior cerebral edema syndrome, is a reversible neurological condition characterized by headache, altered mental status, visual disturbances, seizures, and unconsciousness. It is a clinic-radiological entity with characteristic features on neuroimaging. We present a case of a young antenatal term patient with pre-eclampsia who had no previous history of hypertension. She underwent a cesarean section and in the post-natal period developed sudden bilateral severe vision loss without seizure because of PRES. The visual loss was reversible and improved on systemic and supportive anti-hypertensive patient.

Keywords: Posterior reversible encephalopathy syndrome, reversible, visual outcome, visual symptoms

How to cite this article:
Prasad N, Dubey A, Kumar K. Posterior reversible encephalopathy syndrome: A case report on visual outcome in a patient with pre-eclampsia. Indian J Ophthalmol Case Rep 2023;3:475-7

How to cite this URL:
Prasad N, Dubey A, Kumar K. Posterior reversible encephalopathy syndrome: A case report on visual outcome in a patient with pre-eclampsia. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 1];3:475-7. Available from: https://www.ijoreports.in/text.asp?2023/3/2/475/375037

Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiological syndrome with diverse clinical presentation and characteristic magnetic resonance imaging (MRI) features.^[1] It is a reversible neurological syndrome of acute onset characterized by a range of symptoms such as headache, nausea, altered mental status, visual disturbances, generalized seizures, confusion, unconsciousness, and coma.^[2],[3] The syndrome was first described in 1996 by Hinchey.^[4] PRES has been reported in all age groups but most frequently seen in young or middle age adults with a preponderance toward females.^[1]

There are two leading theories that are associated with PRES.^[1],[3] The first one is hyper-perfusion theory, which includes cerebral hyper-perfusion in response to elevated or fluctuating blood pressure above the auto-regulatory limit, which causes vascular leakage and vasogenic edema, leading to cerebral ischemia.^[1],[3] The second theory is toxic or immunogenic theory, which states that the syndrome is triggered by endothelial dysfunction caused by circulating endogenous or exogenous toxins, which results in increased vascular permeability and edema formation.^[1],[3] The various systemic associations with PRES include acute hypertension, pre-eclampsia, eclampsia, renal disease, vascular disease, auto-immune disease, organ transplantation, exposure to immuno-suppressive and cytotoxic drugs, sepsis, and multi-organ dysfunction syndrome.^[1] The clinical presentation includes elevated arterial blood pressure up to hypertensive emergencies.^[1] Neuroimaging, in particular MRI, typically shows a distinctive parieto-occipital pattern with a symmetric distribution of changes reflecting vasogenic edema.^[5]

Case Report

A young primi gravida on post-natal day 1, following cesarean section at full term, developed sudden onset painless severe diminution of vision in both eyes associated with headache. She had a history of pre-eclampsia and was on irregular anti-hypertensive treatment. There was no history of seizures in the antenatal, natal, or post-natal period. There were no similar complaints or history of hypertension in the past. There was no family history of similar complaints or any chronic illness. General physical examination on post-natal day 1 revealed a blood pressure of 170/110 mmHg. She was conscious and oriented to time, place, and person. Visual acuity in both eyes was counting finger one feet with projection of rays accurate in all four quadrants. The anterior segment of both the eyes was normal. Pupillary reaction was normal; both direct and consensual light reflexes were present. Extra-ocular movements were full and free in all gazes. The intra-ocular pressure in both eyes was 17.3 mmHg, measured using a Schiotz tonometer. The fundus under mydriasis was within physiological limits in both eyes.

With a normal fundus to rule out other causes of severe painless bilateral vision loss in a post-partum patient with pre-eclampsia, MRI brain was performed. MRI brain revealed multi-focal hyper-intensities in subcortical and deep white matter of bilateral parieto-occipital lobes in T2 and fluid-attenuated inversion recovery (FLAIR) images [Figure 1] and [Figure 2]. Keeping the clinical correlation in mind, the history of pre-eclampsia, the rise in blood pressure post-operatively, and the typical MRI picture, the appearance was because of PRES. Control of blood pressure was the main stay of treatment. She was started on the anti-hypertensives hydralazine and labetalol along with other supportive conservative treatment to fasten the stabilization. Adequate hydration and intravenous fluids were given. Maintenance of serum electrolyte and prevention of seizures were given attention. The correction of hypertension was performed cautiously because a rapid reduction in blood pressure can worsen the cytotoxic edema. On follow-up of post-natal day 5, the patient was clinically better. Her blood pressure was reduced to 140/90 mmHg. The best corrected visual acuity in both eyes was 6/60. Extra-ocular movements were full and free. The fundus under mydriasis was within physiological limits in both eyes. On follow-up of post-natal day 10, her blood pressure was 120/80 mmHg. The visual acuity in both eyes was improved to 6/6. Meanwhile, the patient had no episode of seizures anytime. The patient showed marked improvement clinically with control of blood pressure and was discharged in a stable condition without any complications on the 10^th day after delivery.

Figure 1: MRI brain with contrast shows multi-focal patchy T2 hyper-intensities in subcortical and deep white matter of bilateral parieto-occipital lobes and bilateral centrum semiovale

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Figure 2: MRI brain with contrast shows multi-focal patchy FLAIR hyper-intensities in subcortical and deep white matter of bilateral parieto-occipital lobes and bilateral centrum semiovale

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Discussion

PRES is a rare syndrome of central nervous system. Seizures, usually generalized tonic clonic, are often the presenting manifestation in obstetric patients.^[6] Our case has a unique presentation because it occurred without any episode of seizure in a post-partum pre-eclamptic patient. Sudden painless profound visual impairment without seizure can be the presenting complaints of posterior reversible encephalopathy syndrome in patients with eclampsia or pre-eclampsia. Hence, ocular symptoms should be given the priority in evaluating the patients with PRES, especially in the pre-natal or post-natal period. Ophthalmologists should therefore be aware of the entity. Obstetric patients should be given specific attention toward visual disturbances in order to avoid the threatening complications. Blindness in the pre-eclampsia patient is more likely to be associated with cortical etiology.^[7] Early diagnosis of PRES is essential to prevent complications such as cerebral infarction and hemorrhage.^[8] Proper diagnosis requires careful attention to the clinical and radiological features.^[8] An imaging study is also needed to exclude other conditions such as cerebral venous thrombosis or an acute cerebrovascular accident. It is more important in individuals with hypertension, pre-eclampsia, eclampsia, renal disease, vascular disease, auto-immune disease, sepsis, organ transplantation, and multi-organ dysfunction syndrome and those on immuno-suppressive and cytotoxic drugs.^[1] When detected at an early stage, it can lead to reversible changes including visual symptoms.^[9] A high index of suspicion and prompt treatment can reduce morbidity and mortality and pave the path for an early recovery. A sudden onset of profound painless bilateral visual impairment even without seizure, with a normal fundus picture, should be a suspicion toward the syndrome, which should be confirmed on imaging as a characteristic lesion location in parietal and occipital lobes followed by the frontal lobes, the inferior temporal-occipital junction, and the cerebellum.^[1],[5] The abnormalities are seen as hyper-intense T2 and FLAIR images in the parieto-occipital and posterior frontal cortical and subcortical white matter; less commonly, brainstem, basal ganglia, and cerebellum are involved.^[5] We especially want to emphasize that the said syndrome can occur in the post-partum period without seizure. The prognosis is good if promptly recognized and treated. The clinical syndrome usually resolves within a week, whereas the changes seen in MRI resolve over days to weeks.^[9] After appropriate timely treatment, the imaging shows resolution of the lesions unless the condition progresses to complications such as infarction or hemorrhage.^[9] Once complication develops, it leads to visual sequelae such as cortical blindness, homonymous hemianopia, visual neglect, blurred vision, auras, and visual hallucinations. Hence, it is imperative that the syndrome of PRES is correctly and timely recognized as the condition is reversible and potential complications can be avoided with appropriate therapy.^[8]

Conclusion

PRES is not uncommon in pre-eclampsia or eclampsia patients but may be a diagnostic and therapeutic challenge. The syndrome is generally characterized by seizures, but visual impairment occurring in a pre-eclamptic patient should be suspected for PRES even if seizures do not accompany the clinical presentation. Hence, an ophthalmologist should be aware of the uncommon presenting manifestation of the disorder. It is a reversible disorder if diagnosed timely and managed appropriately. Hence, any suspicion of sudden painless diminution of vision in such patients even without seizures should be carefully observed for it. PRES if managed timely can reverse profound bilateral visual impairment. Effective team work and patient counseling are necessary for proper management of the case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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