American Journal of Emergency Medicine xxx (2013) xxx–xxx

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Case Report

Posterior reversible encephalopathy syndrome as the first presentation of chronic kidney disease☆ Abstract Posterior reversible encephalopathy syndrome is a clinicoradiologic syndrome characterized by seizure, headache, nausea, vomiting, altered mental status, visual disturbance, or visual loss, together with radiologic findings. Herein we reported a patient who was a 27-year-old foreign workman brought to the emergency ward with blurred vision in both eyes, neck pain, somnolence, and altered mental status. He had no known medical or family history. On his physical examination, his blood pressure was 190/100 mm Hg and visual acuity of both eyes was decreased (visual acuity = 20/40 for both eyes). Although blood pressure was controlled by intravenous labetalol bolus, his symptoms did not improve. The result of his brain computed tomographic scan was normal, but in the brain magnetic resonance imaging, there were hyperintensities in the occipital lobe and right cerebellar hemisphere, suggesting posterior reversible encephalopathy syndrome. The findings from a complete blood count, biochemical testing, and renal, pelvic and abdominal ultrasound and also the calculated glomerular filtration rate values revealed that all were in favor of chronic kidney disease (hemoglobin level, 10.3 mg/dL; blood urea nitrogen, 90 mg/dL; Cr, 6.8 mg/dL; potassium, 5 mEq/L; sodium, 140 mEq/L; Cockcroft-Gault glomerular filtration rate, 15; weight, 65 kg). The hemodialysis was performed by which the sign and symptoms and radiologic findings resolved within 24 hours. Although PRES is described to be in association with many medical conditions, as mentioned in many available articles, we came to a conclusion that PRES could be the first presentation of a condition such as chronic kidney disease in our case. Posterior reversible encephalopathy syndrome (PRES) also known as hypertensive encephalopathy or reversible posterior leukoencephalopathy is a clinicoradiologic and brain capillary leak syndrome that was first described in 1996 by Hinchey et al [1]. It is characterized by seizure, headache, nausea, vomiting, altered mental status, visual disturbance or visual loss, and freezing of gait, together with radiologic findings [2-8]. Its known etiologies are as follows: acute hypertension, eclampsia, pre-eclampsia, renal disease, sepsis, exposure to immunosuppressant such as cyclosporine A and tacrolimus, and post–renal transplantation [2-5,9]. It is also known that PRES is associated with concurrency of thrombotic thrombocytopenic purpura and adult onset of Still disease [10] and several other medical conditions summarized in Table. We herein report a case of PRES as a first presentation of chronic kidney disease (CKD) in a 27-year-old foreign workman who was ☆ There were no sources for support, and the article has not been presented before.

referred to the emergency ward by his colleague. He had blurred vision, neck pain, somnolence, and altered mental status. All symptoms had occurred on the day before and were aggravated on the day of his admission in the hospital. He had no known medical history and family medical history. On physical examination, his blood pressure was 190/100 mm Hg, and the visual acuity of both eyes was reduced (visual acuity, 20/40 for both eyes). He had no focal neurologic deficit on his physical examination. Cranial computed tomography (CT) was performed, which showed no significant lesion, but in his brain magnetic resonance imaging (MRI), multiple cortical and subcortical foci with T1-weighted images in MRI and T2-weighted images in MRI prolongation were seen predominantly both in the occipital lobe in the supratentorial region, also with the involvement of the right cerebellar hemisphere (Fig). Although his blood pressure was controlled with 10 mg intravenous labetalol bolus (blood pressure, 135/85 mm Hg), the signs and symptoms, together with cranial imaging findings, did not

Table Conditions associated with PRES Reference Liver transplantation Liver injury due to sodium valproate toxicity Hyperammonemia due to chronic liver failure Post–streptococcal glomerulonephritis Steroid therapy Cyclic vomiting Treatment of leukemia Malignancies and chemotherapic agents like L-asparginase and cisplatin Autoimmune lymphoproliferative syndrome Intra-abdominal neurogenic tumors like ganglioneuroma Henoch-Schönlein purpura Hemolytic anemic syndrome Porphyria Addison disease Intravenous immunoglobulin therapy Bone marrow transplantation Nonsteroidal anti-inflammatory drugs Drug-induced acute tubal and interstitial nephritis Sickle cell disease Tumor lysis Atopic dermatitis with group A β-hemolytic streptococcus skin infection Bronchial asthma Interferon-α Intrathecal methotrexate Indinavir and antiretroviral agents Hypercalcemia leading to microvascular spasm or by its direct toxic effect

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Please cite this article as: Forouzanfar M, et al, Posterior reversible encephalopathy syndrome as the first presentation of chronic kidney disease, Am J Emerg Med (2013), http://dx.doi.org/10.1016/j.ajem.2013.11.013

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M. Forouzanfar et al. / American Journal of Emergency Medicine xxx (2013) xxx–xxx

Fig. T2-weighted and fluid-attenuated inversion recovery views of brain MRI of the patient on admission day.

improve. Biochemical testing revealed a blood urea nitrogen level of 90 mg/dL, serum creatinine level of 6.8 mg/dL, serum potassium level of 5 mEq/L, and serum sodium level of 140 mEq/L. The Cockcroft-Gault glomerular filtration rate was 15 (weight, 65 kg). His complete blood count revealed a hemoglobin level of 10.3 mg/ dL. In renal ultrasound, sizes of kidneys were decreased, the parenchymal echogenisity was increased, and corticomedullary differentiations were lost on both sides. No renal stone and hydronephrosis were seen. The findings from pelvic and abdominal ultrasound were normal. With CKD diagnosis, hemodialysis was performed 3 hours after the patient's admission by which his blurred vision, somnolence, and cranial radiologic findings resolved within 24 hours. Currently, the patient, as a case of End Stage Renal Disease, undergoes hemodialysis 3 times a week. The pathogenesis of hypertensive encephalopathy or PRES due to acute hypertension is that the cerebral autoregulation mechanism, over a range of mean arterial blood pressure, preserves constant cerebral blood flow, but as the mean arterial pressure rises, the cerebral autoregulation fails because of arteriolar vasodilatation and

endothelial dysfunction leading to blood–brain barrier disruption and capillary leakage. Although the pathophysiologies of PRES caused by immunosuppressive therapy are not that clear, perhaps vasospasm and reduced tissue perfusion followed by activation of the coagulation cascade and fluid extravagation due to vascular endothelial damage are the causes of it; therefore, cerebral edema and the pathophysiologies of PRES are vasogenic rather than cytogenic edema [2]. The radiologic findings may be seen in an unenhanced CT scan, but the diagnostic and prognostic tool is MRI [4]. Likewise, in our case, there was no significant lesion in the CT scan imaging, but in MRI, the hyperintensity foci in the cortical and subcortical areas were detectable. In both types of imaging, the white matter is more affected rather than the cortex because it is less tightly packed. The radiologic findings are focal usually asymmetrical vasogenic edema of white matter involving bilateral parieto-occipital lobes. Usually, posterior cranial fossa involvement is uncommon [2]. Apart from the mentioned affected parts, other cortical areas and deep gray nuclei can also be involved [6]. In an unenhanced CT of the brain, the

Please cite this article as: Forouzanfar M, et al, Posterior reversible encephalopathy syndrome as the first presentation of chronic kidney disease, Am J Emerg Med (2013), http://dx.doi.org/10.1016/j.ajem.2013.11.013

M. Forouzanfar et al. / American Journal of Emergency Medicine xxx (2013) xxx–xxx

involvement of subcortical white matter and cortical areas with low attenuation is seen. In MRI, vasogenic edema is seen as hyperintensities on T2-weighted imaging sequences [4]. Diffusion-weighted MRI sequences are helpful in distinguishing venous infarction and mitochondrial encephalopathy from PRES lesions by calculating the apparent diffusion coefficient (ADC). In the former, the ADC value is increased as opposed to decreased ADC values in the latter, which are seen as restricted diffused lesions [2]. Also for the detection of the degree of cortical involvement and the extension of hyperintense lesions, fluid-attenuated inversion recovery MRI sequences can be used [2]. The differential diagnoses of PRES are as follows: posterior cerebral artery infarction in which, in contrast to PRES, the calcarine and paramedian parts of the occipital lobe are also involved; venous thrombosis; vasculitis, demyelinating disorders; and encephalitis [2]. Although reversible posterior leukoencephalopathy is reversible by definition, it is not always reversible and not always involved in the posterior parts of the brain. Depending on etiology, most of the cases resolve within 1 to 2 weeks of eliminating the causative factor, but PRES can also lead to secondary complications such as status epilepticus, intracranial hemorrhage, massive ischemic infarction, mesial temporal sclerosis, and death. Also, as noted above, apart from white matter of parieto-occipital lobes, the cortical areas and deep gray nuclei can also be involved [2,6]. In conclusion, according to this case, we should keep in mind that apart from the association of PRES with several medical conditions including CKD, it can be the first presentation of CKD.

Mohammadmahdi Forouzanfar MD Department of Emergency Medicine Shohadaye Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

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Pauline Haroutunian MD Department of Medicine Shahid Beheshti University of Medical Sciences, Tehran, Iran E-mail address: [email protected] Alireza Baratloo MD Behrooz Hashemi MD Department of Emergency Medicine Shohadaye Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran http://dx.doi.org/10.1016/j.ajem.2013.11.013 References [1] Hinchey J, Chaves C, Appiggnani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494–500 [PubMed]. [2] Chandramohan V, Nagarajan VP, Sathyamoorthi MS, et al. Posterior reversible encephalopathy syndrome in a child with autoimmune lymphoproliferative syndrome. J Pediatr Neurosci 2012;7(3):221–4. [3] Mettananda S, Fernando AD, Ginige N. Posterior reversible encephalopathy syndrome in a survivor of valproate-induced acute liver failure. J Med Case Rep 2013;7:144. [4] Puppala S, Hurihan MD. A pressing case of transient blindness. Br J Radiol 2005;78: 967–8. [5] Golombeck SK, Wessig C, Monoranu C-M, et al. Fatal atypical reversible posterior leukoencephalopathy syndrome. J Med Case Rep 2013;7:14. [6] Tungkasaereerak C, Phanthumchinda K. Reversible posterior leukoencephalopathy syndrome. J Med Assoc Thai 2008;91(3):427–32. [7] Nakabou M, Kai T, Maeshima T, et al. Hypertensive encephalopathy in patients with chronic renal failure caused by stopping antihypertensive agents. Clin Exp Nephrol 2010;14:256–62. [8] Nakajima A, Ueno Y, Shimura H, et al. Acute transient freezing of gait in a patient with posterior reversible encephalopathy syndrome. BMC Neurol 2013;13(1):79. [9] Alexander S, David VG, Varughese S, et al. Posterior reversible encephalopathy syndrome in a renal allograft recipient. Indian J Nephrol 2013;23(2):137–9. [10] Khobraqade AK, Choqle AR, Ram RP, et al. Reversible posterior leukoencephalopathy syndrome in a case of adult onset Still’s disease with concurrent thrombotic thrombocytopenic purpura. J Assoc Physicians India 2012;60:59–62.

Please cite this article as: Forouzanfar M, et al, Posterior reversible encephalopathy syndrome as the first presentation of chronic kidney disease, Am J Emerg Med (2013), http://dx.doi.org/10.1016/j.ajem.2013.11.013

Posterior reversible encephalopathy syndrome as the first presentation of chronic kidney disease.

Posterior reversible encephalopathy syndrome is a clinicoradiologic syndrome characterized by seizure, headache, nausea, vomiting, altered mental stat...
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