J Neurocrit Care.  2024 Dec;17(2):62-66. 10.18700/jnc.240019.

A fatal case of malignant posterior reversible encephalopathy syndrome in the setting of status epilepticus: a case report

• Unavane O
• Raut TP
• Parajiya L
• Sadarangani AR

• KMID: 2563023
• DOI: http://doi.org/10.18700/jnc.240019

Abstract

Background
Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible neurotoxicity. The symptoms include headache, altered consciousness, seizures, visual disturbances, and focal neurological deficits. The severe variant, known as malignant PRES, is rare and involves diffuse cerebral edema, leading to irreversible damage. Chronic kidney disease (CKD) with impaired blood pressure regulation is a known risk factor for malignant PRES.
Case report
We report the case of a patient with CKD who presented with hypertensive crisis and later developed malignant PRES in the background of status epilepticus refractory to antiepileptic treatment.
Conclusions
This case highlights the importance of considering malignant PRES in hypertensive crises and emphasizes the need for further research to elucidate its pathophysiology and develop effective treatment strategies.

Keyword

Case report; Posterior reversible encephalopathy syndrome; Hypertensive crisis; Status epilepticus

Figure

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  Fig. 1. Electroencephalogram (EEG) showing generalized polyspikes. EEG record which was done started at 3-Hz generalized spike, polyspike and wave pattern lasting for 2 minutes followed by background suppression.

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  Fig. 2. Serial computed tomography (CT) scan images. (A) Initial CT scan. The CT image did not reveal any significant edema or hemorrhage. (B) CT scan performed 12 hours after initial CT scan. The image revealed extensive diffuse parenchymal edema with notable cerebral white matter edema resulting in basal cisternal and lateral ventricular effacement. The edema was more in the right cerebral hemisphere resulting in a midline shift of 9 mm from right-to-left. Intracranial flow is maintained. (C) CT scan performed 3 days after initial CT scan. Repeat CT brain showed significant reduction in the diffuse cerebral edema resulting in decrease in cerebral sulcal and ventricular effacement with decrease in midline shift (now measures 6 mm to the left) and uncal herniation with reduction of mass-effect on the midbrain and upper pons.

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  Fig. 3. Magnetic resonance imaging image showing features of malignant posterior reversible encephalopathy syndrome.

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  Fig. 4. Magnetic resonance imaging image showing Duret hemorrhages.

Reference

1. Kelly DM, Ademi Z, Doehner W, Lip GY, Mark P, Toyoda K, et al. Chronic kidney disease and cerebrovascular disease: consensus and guidance from a KDIGO Controversies Conference. Stroke. 2021; 52:e328–46.

2. Matsuki H, Mandai S, Shiwaku H, Koide T, Takahashi N, Yanagi T, et al. Chronic kidney disease causes blood-brain barrier breakdown via urea-activated matrix metalloproteinase-2 and insolubility of tau protein. Aging (Albany NY). 2023; 15:10972–95.

3. Kong X, Wen JQ, Qi RF, Luo S, Zhong JH, Chen HJ, et al. Diffuse interstitial brain edema in patients with end-stage renal disease undergoing hemodialysis: a tract-based spatial statistics study. Medicine (Baltimore). 2014; 93:e313.

4. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol. 2008; 65:205–10.

5. Prescriber Update. Posterior reversible (leuko) encephalopathy syndrome (PRES): increasingly linked to medicines [Internet]. Govt.nz; 2017 [cited 2024 May 24]. Available from: https://www.medsafe.govt.nz/profs/PUArticles/March2017/PERSLinkedtoMedicines.htm.

6. Magsi S, Zafar A. Malignant posterior reversible encephalopathy syndrome: an exacting challenge for neurocritical care physicians. Neurohospitalist. 2017; 7:196–9.

7. Navinan MR, Subasinghe CJ, Kandeepan T, Kulatunga A. Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report. BMC Res Notes. 2014; 7:89.

8. Akins PT, Axelrod Y, Silverthorn JW, Guppy K, Banerjee A, Hawk MW. Management and outcomes of malignant posterior reversible encephalopathy syndrome. Clin Neurol Neurosurg. 2014; 125:52–7.

9. Martins WA, Marrone LC. Malignant posterior reversible encephalopathy syndrome: a case of posterior irreversible encephalopathy syndrome. J Clin Neurol. 2016; 12:236–7.

10. Palmer BF. Disturbances in renal autoregulation and the susceptibility to hypertension-induced chronic kidney disease. Am J Med Sci. 2004; 328:330–43.

11. Rodriguez W, Tseng S, Pastrana F, Wang F. Case report: a fatal case of malignant posterior reversible encephalopathy syndrome in the setting of diabetic ketoacidosis. Cureus. 2023; 15:e45218.

12. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996; 334:494–500.