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LETTER TO EDITOR |
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Year : 2012 | Volume
: 22
| Issue : 2 | Page : 153-154 |
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Posterior reversible encephalopathy syndrome in minimal change disease
G Swarnalatha, R Ram, B. H. S. Pai, KV Dakshinamurty
Nizam's Institute of Medical Sciences, Punjagutta, Hyderbad, India
Date of Web Publication | 12-Jun-2012 |
Correspondence Address: K V Dakshinamurty Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-4065.97153
How to cite this article: Swarnalatha G, Ram R, Pai B, Dakshinamurty K V. Posterior reversible encephalopathy syndrome in minimal change disease. Indian J Nephrol 2012;22:153-4 |
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.
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, [3],[4] 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.  |
2. | 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.  |
3. | Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: Prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 2002;23:1038-48  |
4. | Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Shishido S, Asanuma H, et al. Posterior reversible encephalopathy syndrome in children: Its high prevalence and more extensive imaging findings. Am J Kidney Dis 2006;48:231-8  |
5. | Ikeda M, Ito S, Hataya H, Honda M, Anbo K. Reversible posterior leukoencephalopathy in a patient with minimal-change nephrotic syndrome. Am J Kidney Dis 2001;37:E30.  [PUBMED] [FULLTEXT] |
6. | Pearson ER, D'Souza RJ, Hamilton-Wood C, Nicholls AJ, Beaman M. Hypertensive encephalopathy and nephrotic syndrome: A possible link? Nephrol Dial Transplant 1999;14:1750-2.  [PUBMED] [FULLTEXT] |
[Figure 1]
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