PRES (Posterior Reversible Encephalopathy Syndrome): A case series of PRESsing concern

Anne Prewina Gurushekar

Consultant Pediatrician, 1win, Tirunelveli

Abstract

These are brief events in the life of two children who presented with features of Posterior Reversible Encephalopathy Syndrome (PRES), albeit with two completely different histories.

Case 1

A 13-year-old girl diagnosed with SLE Nephritis 4 years back, was on treatment with immunosuppressive drugs (cyclophosphamide, tacrolimus), steroids and anti-hypertensives. She was doing well on regular medications until one day she developed a severe headache lasting almost 24 hours followed by unresponsiveness. She initially went to a nearby hospital and was then referred here. On arrival she was unconscious and her BP was 190/140 mmHg.

An urgent CT brain showed patchy hypodense areas in bilateral parietal, bilateral occipito – temporal and right cerebellar regions, suggestive of PRES. She was intubated on arrival, stabilized and her BP was gradually brought under control over the next 48 hr with IV infusions of Labetolol and other anti-hypertensives. She developed seizures on the first day which was controlled. Her immunosuppressive drugs were stopped temporarily.

Her sensorium improved and she was extubated within 48 hours. Initially, she had intermittent episodes of headache and visual disturbances. She also had memory lapses of several events over the last four hours and had a brief episode of delirium.

The Nephrologist, Rheumatologist and Neurologist were involved throughout in her care. Over ten days, she gradually improved. MRI confirmed findings of PRES. She was headache-free but surprisingly, the memory loss of several past events remained even after discharge.

Fig (1): CT brain showed patchy hypodense areas

Case 2

Our second patient, a 10 year aged boy was a developmentally normal and well- thriving child who had some facial swelling the previous week for which he was treated outside. He was suspected to have allergy. He complained of headache for a few days following which he developed an episode of seizure and was then brought here.

On arrival, his seizures were brought under control. His BP was found to be high (160/100 mm Hg) and he was treated with anti-hypertensives. A CT taken showed features suggestive of PRES which was later confirmed on MRI. Urine for routine analysis was sent immediately and was found to have albuminuria with RBC’s present. He had a history of decrease in urine output which the parents did not think significant at the time. His C3 levels were low and ASO titre was positive. He had a history of impetigo the previous month. This clinched the diagnosis of post infectious glomerulonephritis with PRES. His BP was brought under control. He also had weakness of his right upper limb which resolved over next 48 hr. His urine output improved and his anti – hypertensives were tapered over 1 week. He was followed by the critical care, neurology and nephrology team. He improved completely and has since been on regular follow up.

Fig (2): Posterior Reversible Encephalopathy Syndrome

Discussion

Posterior reversible encephalopathy syndrome (PRES) is a clinical condition that is characterized by intense headache, visual disturbances, seizures and neurological deficits which are attributed to the vasogenic edema that occurs in the posterior cerebral cortex involving the occipital and parietal lobes. Endothelial dysfunction is a key element in PRES, pathogenesis particularly in lupus patients.

PRES commonly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs and can be complicated with vasculopathy, infarction or hemorrhage . Patients with SLE are more likely to experience headache due to vascular causes such as cerebral venous sinus thrombosis, stroke, reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, and vasculitis. Meningo – encephalitis, intracranial neoplasms, and intracranial hypertension or hypotension may also be a cause of headache in these patients and need to be ruled out. PRES is now being increasingly recognized in association with autoimmune disorders.

Although PRES was initially described as benign, wholly reversible and with a good prognosis, its mortality is around 19%, and about 44% of patients are left with varying degrees of functional impairments. Follow-up imaging may find residual structural lesions in 40% of cases.

Conclusion

However, both children had different causes. The quick control of seizures, anti hypertensives and early diagnosis played a key role in successful management. Clinical and radiographic resolution of abnormalities within 1-4 weeks was also typically seen.

Reference

Ritasman Baisya, Phani Kumar Devarasetti, Ramakrishna Narayanan, Liza Rajasekhar. Posterior reversible encephalopathy syndrome in juvenile lupus- a case series and literature review. Department of Clinical Immunology and Rheumatology, 28605Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, India.
M L Ishimori 1, B D Pressman, D J Wallace, M H Weisman. Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE. Division of Rheumatology, Cedars-Sinai Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Javier Merayo-Chalico, Elia Apodaca, Ana Barrera-Vargas, Jorge Alcocer-Varela, Iris Colunga-Pedraza, et al. Clinical outcomes and risk factors for posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a multicentric case–control study. BMJ journal of Neurology, Neurosurgery and Psychiatry
James D Triplett, Mansur Amirovich Kutlubaev, Allan G Kermode, Todd Hardy. Posterior reversible encephalopathy syndrome (PRES): diagnosis and management. Department of Neurology, Concord Hospital, Concord, NSW 2139.

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PRES (Posterior Reversible Encephalopathy Syndrome): A case series of PRESsing concern