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The Case ∣ Glomerulonephritis and altered mental status
make your diagnosis
http://www.kidney-international.org & 2007 International Society of Nephrology Kidney International (2007) 72, 1413–1414. doi:10.1038/sj.ki.5002542
The Case | Glomerulonephritis and altered mental status R Ram1, G Swarnalatha1, N Prasad1, KV Dakshinamurty1 1
Department of Nephrology, Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, India
Correspondence: K.V. Dakshinamurty, Department of Nephrology, Nizam’s Institute of Medical Sciences (NIMS), Punjagutta, Hyderabad 500082, India. E-mail: [email protected]
Figure 1 | MRI of brain.
A 7-year-old boy presented to our hospital with abrupt onset of facial puffiness and swelling of feet. He also complained of oliguria, smoky urine, headache, confusion, and blurring of vision. Five days prior to hospitalization, he was noted to have sore throat and fever. On examination, his blood pressure was 250/140 mm Hg. Laboratory investigations revealed serum creatinine 1.9 mg dl 1, blood urea 133 mg dl 1, serum sodium 148 mEq l 1, serum potassium 3.8 mEq l 1, and hemoglobin 12.5 g dl 1. There were no schistocytes in the peripheral smear. His urine revealed plenty of dysmorphic red blood cells and a red blood cell cast. His
antistreptolysin titer was elevated at 800 IU ml 1 (normal: o200 IU ml 1). Complement C3 was 12 mg dl 1 (normal: 55–120 mg dl 1) and C4 was 16 mg dl 1 (normal: 10–40 mg dl 1). Antinuclear antibody was negative. Lactate dehydrogenase was 175 IU ml 1. The next morning, his serum creatinine has increased to 2.9 mg dl 1. His blood pressure required nitroglycerin infusion and four other antihypertensives to control. A renal biopsy was done on the same day. The blurring of vision has persisted so also the confusion. A magnetic resonance imaging (MRI) of brain was performed to investigate the cause for his abnormal mental status (Figure 1).
What characteristic abnormality is seen on the MRI? SEE NEXT PAGE FOR ANSWERS Kidney International (2007) 72, 1413–1414
1413
make your diagnosis
R Ram: A child with glomerulonephritis
The Diagnosis | Posterior Reversible Encephalopathy Syndrome (1) MRI of brain reveals symmetrical areas of hyperintensities in the bilateral parietal and occipital lobes. (2) The clinical diagnosis is the posterior reversible encephalopathy syndrome (PRES) associated with acute postinfectious glomerulonephritis. Renal biopsy was consistent with acute postinfectious glomerulonephritis. After a week of salt-free diet, diuretics, and antihypertensive therapy, his renal function had normalized. On repeat MRI, 2 weeks later, hyperintensities had resolved. PRES is a recently described, clinicoradiologic entity characterized by headache, altered consciousness, visual disturbances, seizures, and characteristic subcortical edema without infarction in a relative symmetric pattern, which predominantly involves parietal and occipital lobes.1 Hypertensive encephalopathy, renal failure, cyclosporine and tacrolimus therapy, and eclampsia are major factors associated with PRES.2 In our patient, PRES was presumed to have been associated with both hypertension and renal failure. There has been only one other report of PRES associated with acute post streptococcal glomerulonephritis.3 The pathogenesis of PRES is unclear but appears to be associated with altered cerebral autoregulation and endothelial dysfunction.1 Hinchey et al.2 suggested that PRES represented a localized manifestation of hypertensive
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encephalopathy occurring secondary to hypertensive crisis. The degree of hypertension required to induce encephalopathy depends on the baseline pressure. Lethargy and somnolence are often the first symptoms noted. Visual perception abnormalities are invariably present. The role of neuroimaging is to establish the initial diagnosis and exclude other causes of neurological features. Noncontrast computerized tomography is sufficient to make the diagnosis in the proper clinical setting. MRI features are characteristic.4 Since the lesions in PRES are typically in subcortical and cortical locations near brain surfaces, detection with T2-weighted imaging is difficult in mild cases, yet Fluid-Attenuated inversion recovery (FLAIR) sequence can suppress the signal of the adjacent cerebrospinal fluid, rendering lesions of PRES conspicuous.1 Diffusion-weighted imaging can differentiate this condition from ischemic/cytotoxic edema.4 REFERENCES 1.
2. 3.
4.
Casey SO, Sampaio RC, Michel E et al. Posterior reversible encephalopathy syndrome: utility of fluid attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000; 21: 1199–1206. Hinchey J, Chaves C, Appignani B et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494–500. Soyalu A, Kavukc¸u S, Tu¨rkmen M et al. Posterior leukoencephalopathy syndrome in poststreptococcal acute glomerulonephritis. Pediatr Nephrol 2001; 16: 601–6033. Das CJ, Sethi A. Posterior reversible encephalopathy syndrome. Indian Pediatr 2006; 43: 657–658.
Kidney International (2007) 72, 1413–1414
http://www.kidney-international.org & 2007 International Society of Nephrology Kidney International (2007) 72, 1413–1414. doi:10.1038/sj.ki.5002542
The Case | Glomerulonephritis and altered mental status R Ram1, G Swarnalatha1, N Prasad1, KV Dakshinamurty1 1
Department of Nephrology, Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, India
Correspondence: K.V. Dakshinamurty, Department of Nephrology, Nizam’s Institute of Medical Sciences (NIMS), Punjagutta, Hyderabad 500082, India. E-mail: [email protected]
Figure 1 | MRI of brain.
A 7-year-old boy presented to our hospital with abrupt onset of facial puffiness and swelling of feet. He also complained of oliguria, smoky urine, headache, confusion, and blurring of vision. Five days prior to hospitalization, he was noted to have sore throat and fever. On examination, his blood pressure was 250/140 mm Hg. Laboratory investigations revealed serum creatinine 1.9 mg dl 1, blood urea 133 mg dl 1, serum sodium 148 mEq l 1, serum potassium 3.8 mEq l 1, and hemoglobin 12.5 g dl 1. There were no schistocytes in the peripheral smear. His urine revealed plenty of dysmorphic red blood cells and a red blood cell cast. His
antistreptolysin titer was elevated at 800 IU ml 1 (normal: o200 IU ml 1). Complement C3 was 12 mg dl 1 (normal: 55–120 mg dl 1) and C4 was 16 mg dl 1 (normal: 10–40 mg dl 1). Antinuclear antibody was negative. Lactate dehydrogenase was 175 IU ml 1. The next morning, his serum creatinine has increased to 2.9 mg dl 1. His blood pressure required nitroglycerin infusion and four other antihypertensives to control. A renal biopsy was done on the same day. The blurring of vision has persisted so also the confusion. A magnetic resonance imaging (MRI) of brain was performed to investigate the cause for his abnormal mental status (Figure 1).
What characteristic abnormality is seen on the MRI? SEE NEXT PAGE FOR ANSWERS Kidney International (2007) 72, 1413–1414
1413
make your diagnosis
R Ram: A child with glomerulonephritis
The Diagnosis | Posterior Reversible Encephalopathy Syndrome (1) MRI of brain reveals symmetrical areas of hyperintensities in the bilateral parietal and occipital lobes. (2) The clinical diagnosis is the posterior reversible encephalopathy syndrome (PRES) associated with acute postinfectious glomerulonephritis. Renal biopsy was consistent with acute postinfectious glomerulonephritis. After a week of salt-free diet, diuretics, and antihypertensive therapy, his renal function had normalized. On repeat MRI, 2 weeks later, hyperintensities had resolved. PRES is a recently described, clinicoradiologic entity characterized by headache, altered consciousness, visual disturbances, seizures, and characteristic subcortical edema without infarction in a relative symmetric pattern, which predominantly involves parietal and occipital lobes.1 Hypertensive encephalopathy, renal failure, cyclosporine and tacrolimus therapy, and eclampsia are major factors associated with PRES.2 In our patient, PRES was presumed to have been associated with both hypertension and renal failure. There has been only one other report of PRES associated with acute post streptococcal glomerulonephritis.3 The pathogenesis of PRES is unclear but appears to be associated with altered cerebral autoregulation and endothelial dysfunction.1 Hinchey et al.2 suggested that PRES represented a localized manifestation of hypertensive
1414
encephalopathy occurring secondary to hypertensive crisis. The degree of hypertension required to induce encephalopathy depends on the baseline pressure. Lethargy and somnolence are often the first symptoms noted. Visual perception abnormalities are invariably present. The role of neuroimaging is to establish the initial diagnosis and exclude other causes of neurological features. Noncontrast computerized tomography is sufficient to make the diagnosis in the proper clinical setting. MRI features are characteristic.4 Since the lesions in PRES are typically in subcortical and cortical locations near brain surfaces, detection with T2-weighted imaging is difficult in mild cases, yet Fluid-Attenuated inversion recovery (FLAIR) sequence can suppress the signal of the adjacent cerebrospinal fluid, rendering lesions of PRES conspicuous.1 Diffusion-weighted imaging can differentiate this condition from ischemic/cytotoxic edema.4 REFERENCES 1.
2. 3.
4.
Casey SO, Sampaio RC, Michel E et al. Posterior reversible encephalopathy syndrome: utility of fluid attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000; 21: 1199–1206. Hinchey J, Chaves C, Appignani B et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494–500. Soyalu A, Kavukc¸u S, Tu¨rkmen M et al. Posterior leukoencephalopathy syndrome in poststreptococcal acute glomerulonephritis. Pediatr Nephrol 2001; 16: 601–6033. Das CJ, Sethi A. Posterior reversible encephalopathy syndrome. Indian Pediatr 2006; 43: 657–658.
Kidney International (2007) 72, 1413–1414