CASE REPORT Reversible Posterior Leukoencephalopathy in a Patient With Minimal-Change Nephrotic Syndrome Masahiro Ikeda, MD, Shuichi Ito, MD, Hiroshi Hataya, MD, Masataka Honda, MD, and Kazutoshi Anbo, MD ● A 9-year-old boy with nephrotic syndrome was transferred to our hospital because of acute renal failure and disturbance of consciousness after high-dose methylprednisolone therapy. He developed severe headache, visual disturbance, and generalized seizures. Brain computed tomography (CT) scan revealed multiple, bilateral, low- density areas in the parieto-occipital lobes. Magnetic resonance imaging (MRI) disclosed a high signal intensity area on T2-weighted images and a low signal intensity area on T1-weighted images in the same lesion. Follow-up brain CT scan and MRI, 2 weeks after the first studies, showed complete resolution of the abnormal lesions, which suggested the diagnosis of reversible posterior leukoencephalopathy syndrome (RPLS). Hypertension and high- dose methylprednisolone administration to the patient in the nephrotic state may be causes of this uncommon syndrome in this case. This is the first report of RPLS in nephrotic syndrome with hypertension not associated with cyclosporine administration. © 2001 by the National Kidney Foundation, Inc. INDEX WORDS: Leukoencephalopathy; nephrotic syndrome; hypertension; methylprednisolone; convulsion; vi- sual disturbance; magnetic resonance imaging (MRI); computed tomography (CT). R EVERSIBLE POSTERIOR leukoencepha- lopathy syndrome (RPLS) is a rare compli- cation of nephrotic syndrome, exclusively associ- ated with cyclosporine administration in previous reports. We report here a patient with minimal- change nephrotic syndrome who developed RPLS without cyclosporine administration. CASE REPORT A 9-year-old boy with nephrotic syndrome was trans- ferred to our hospital because of acute renal failure and disturbance of consciousness on January 16, 1999. The patient had no previous history of seizures or febrile convul- sions. He had developed generalized edema and had been admitted to the previous hospital on January 5. Albumin and diuretics were administered, and oral prednisolone (2 mg/ kg/d) was started, then from January 13 through January 15 methylprednisolone pulse therapy (30 mg/kg/d for 3 days) was given. The patient was normotensive, and there were no signs of dehydration at the time the pulse therapy was administered. The patient’s renal function deteriorated gradu- ally, serum creatinine and blood urea nitrogen levels rose to 2.0 mg/dL and 119 mg/dL, and he became drowsy, so he was transferred to our hospital. On admission, the patient’s daily urinary protein excretion was greater than 10 g, serum total protein level was 3.4 g/dL, albumin was 1.9 g/dL, and total cholesterol was 569 mg/dL. He showed moderate hypertension (145/85 mm Hg) and bradycardia (heart rate, 45 beats/min). The patient com- plained of severe headache, which was followed by a gener- alized convulsion, with his eyes deviated to the right side, for 3 minutes on the 5th day of admission, then he com- plained of blurred vision and visual hallucinations on the 7th hospital day (“a strange bear, and his father, on the ceiling”). Blood glucose, electrolytes, bicarbonate, serum comple- ment, and complete blood count were all normal. Cerebrospinal fluid examination showed no abnormal- ity, with negative testing for myelin basic protein and oligoclonal bands. Brain computed tomography (CT) scan performed on the day of admission revealed multiple, bilateral, low-density areas in the parieto-occipital lobes. Magnetic resonance imaging (MRI) showed a high signal intensity area on T2-weighted images and a low signal intensity area on T1-weighted images in the same lesion (Fig 1). The electroencephalogram showed sharp waves in the left occipital area. The anticonvulsant valproate was started immediately, but the patient developed a brief episode of loss of consciousness with a blank stare on the 24th day of admission. Follow-up brain CT scan and MRI 2 weeks after the first studies showed complete resolution of the abnormal lesions in bilateral parieto-occipital lobes, which was compatible with the diagnosis of RPLS (Fig 1). The patient’s consciousness became clear gradually, and he developed no additional episodes of convulsions. The patient’s renal function recovered completely, with disappearance of proteinuria on the 19th hospital day. From the Division of Pediatric Nephrology, Tokyo Metro- politan Kiyose Children’s Hospital; and Department of Pedi- atrics, Nippon Medical School, Tokyo, Japan. Received August 7, 2000; accepted in revised form Decem- ber 22, 2000. Address reprint requests to Masahiro Ikeda, MD, Division of Pediatric Nephrology, Tokyo Metropolitan Kiyose Chil- dren’s Hospital, 1-3-1 Umezono, Kiyose, Tokyo 204-8567, Japan. E-mail: m.ikeda@chp.kiyose.tokyo.jp © 2001 by the National Kidney Foundation, Inc. 1523-6838/01/3704-0030$35.00/0 doi:10.1053/ajkd.2001.22867 American Journal of Kidney Diseases, Vol 37, No 4 (April), 2001: E30 1