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Letters to Editor
22 (
2
); 153-154
doi:
10.4103/0971-4065.97153

Posterior reversible encephalopathy syndrome in minimal change disease

Nizam's Institute of Medical Sciences, Punjagutta, Hyderbad, India
Address for correspondence: Dr. K. V. Dakshinamurty, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India. E-mail: kvdm1954@gmail.com
Licence

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Disclaimer:
This article was originally published by Medknow Publications & Media Pvt Ltd and was migrated to Scientific Scholar after the change of Publisher.

Sir,

An 11-year-old girl was being treated elsewhere from the age of 5 years for nephrotic syndrome. She received prednisolone 30 mg per day until remission followed by tapering. She had several relapses during the last 5 years, and presented to us in nephrotic state. Her pulse rate and blood pressure were 78 bpm and 90/70 mmHg, respectively. Systemic examination was unremarkable. Investigations revealed blood urea: 12 mg/dL, serum creatinine: 0.7 mg/dL, serum proteins: 4.7 g/dL, serum albumin: 2.1 g/dL, 24 h urine protein: 4.5 g, serum cholesterol: 450 mg/dL, serum LDL: 267 mg/dL, serum HDL 60 mg/dL, serum VLDL: 158 mg/dL, serum triglycerides 615 mg/dL, hemoglobin: 12 g/dL. Renal biopsy revealed 11 glomeruli, and findings were consistent with minimal change. She received 65 mg of prednisolone in three divided doses per day according to the ISKDC protocol.[1] After 3 weeks, she presented with multiple episodes of generalized tonic clonic seizures. She was afebrile, pulse rate was 160 bpm, blood pressure was 110/60 mmHg. Glasgow coma scale was 7/15. Cerebrospinal fluid analysis revealed 3 cells/hpf, glucose: 56 mg/dL, protein: 15 mg/dL. MRI brain showed bilateral asymmetrical T2, FLAIR hyperintense lesions in cortical and subcortical location of parieto-occipital, temporal lobes, bilateral thalami, and cerebellum suggestive of posterior reversible encephalopathy syndrome (PRES)[Figure 1]. She was treated with antiepileptics and the dose of prednisolone was reduced to half. She recovered completely in 48 h.

Figure 1
MRI brain: Posterior reversible encephalopthy syndrome

PRES, originally termed reversible posterior leukoencephalopathy syndrome[2] presents with headache, seizures, visual changes, altered mental status, and occasionally focal neurologic signs.[3] CT and MR imaging typically show symmetrically distributed areas of vasogenic oedema predominantly within the territories of the posterior circulation.[3]

PRES is seen with a heterogeneous group of disorders.[3] In renal disorders, it is reported with hemolytic the uremic syndrome, thrombotic thrombocytopenic purpura, as a complication of Cyclosporine and tacrolimus,[34] and acute poststreptococcal nephritis[4] In patients with the nephrotic syndrome, the risk factors are administrationof cyclosporine, tacrolimus,[4] methylprednisolone,[5] hypertension and renal insufficiency.

However, nephrotic syndrome itself could be considered a predisposing condition for developing PRES in both adults and children.[6] The key pathophysiological process of PRES is vasogenic edema.[2] due to decreased intravascular oncotic pressure, increased permeability of intracerebral capillaries, and fluid overload. Drugs such as cyclosporine, tacrolimus, and methylprednisolone may induce vasogenic oedema by alterating sympathetic flow, cyclosporine-mediated release of endothelin, or endothelial dysfunction, while hypertension may also induce oedema due to autoregulation failure of the cerebral blood flow.

References

  1. Report of International Study of Kidney Disease in Children: The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr. 1981;98:561-4.
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  2. , , , , , , . A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334:494-500.
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  3. , , , . Posterior reversible encephalopathy syndrome: Prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002;23:1038-48.
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  4. , , , , , , . Posterior reversible encephalopathy syndrome in children: Its high prevalence and more extensive imaging findings. Am J Kidney Dis. 2006;48:231-8.
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  5. , , , , , . Reversible posterior leukoencephalopathy in a patient with minimal-change nephrotic syndrome. Am J Kidney Dis. 2001;37:E30.
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  6. , , , , , . Hypertensive encephalopathy and nephrotic syndrome: A possible link? Nephrol Dial Transplant. 1999;14:1750-2.
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