Abstract
Background
Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.
Objective
We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.
Materials and methods
We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.
Results
Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.
Conclusion
MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.
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References
O’Hara McCoy H (2008) Posterior reversible encephalopathy syndrome: an emerging clinical entity in adult, pediatric, and obstetric critical care. J Am Acad Nurse Pract 20:100–106
Baizabal-Carvallo JF, Barragan-Campos HM, Padilla-Aranda HJ et al (2009) Posterior reversible encephalopathy syndrome as a complication of acute lupus activity. Clin Neurol Neurosurg 111:359–363
Punaro M, Abou-Jaoude P, Cimaz R et al (2007) Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus. Lupus 16:576–579
Lehman T (2002) Systemic lupus erythematosus in childhood and adolescence. In: Wallace DJ (ed) Dubois’ Lupus Erythematosus, pp 864–884
Sacks JJ, Helmick CG, Luo YH et al (2007) Prevalence of and annual ambulatory health care visits for pediatric arthritis and other rheumatologic conditions in the United States in 2001–2004. Arthritis Rheum 57:1439–1445
Tokano Y, Morimoto S, Amano H et al (2005) The relationship between initial clinical manifestation and long-term prognosis of patients with systemic lupus erythematosus. Mod Rheumatol 15:275–282
The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 42:599–608
Brey RL (2007) Neuropsychiatric lupus: clinical and imaging aspects. Bull NYU Hosp Jt Dis 65:194–199
Bodani M, Kopelman MD (2003) A psychiatric perspective on the therapy of psychosis in systemic lupus erythematosus. Lupus 12:947–949
Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR 29:1036–1042
Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR 29:1043–1049
Onder AM, Lopez R, Teomete U et al (2007) Posterior reversible encephalopathy syndrome in the pediatric renal population. Pediatr Nephrol 22:1921–1929
Wirrell EC, Hamiwka LD, Hamiwka LA et al (2007) Acute glomerulonephritis presenting with PRES: a report of 4 cases. Can J Neurol Sci 34:316–321
Morris EB, Laningham FH, Sandlund JT et al (2007) Posterior reversible encephalopathy syndrome in children with cancer. Pediatr Blood Cancer 48:152–159
Khademian Z, Speller-Brown B, Nouraie SM et al (2009) Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer 52:373–375
Lucchini G, Grioni D, Colombini A et al (2008) Encephalopathy syndrome in children with hemato-oncological disorders is not always posterior and reversible. Pediatr Blood Cancer 51:629–633
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725
Brandt JT, Triplett DA, Alving B et al (1995) Criteria for the diagnosis of lupus anticoagulants: an update. On behalf of the Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the ISTH. Thromb Haemost 74:1185–1190
Magnano MD, Bush TM, Herrera I et al (2006) Reversible posterior leukoencephalopathy in patients with systemic lupus erythematosus. Semin Arthritis Rheum 35:396–402
Ishimori ML, Pressman BD, Wallace DJ et al (2007) Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE? Lupus 16:436–443
Acknowledgement
Dr. Muscal’s work was supported by an ACR/LRI Lupus Investigator Fellowship Award, NIH Pediatric Research Loan Repayment Program, and NIH NICHD Child Health Research Career Development Award (K12 HD41648).
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Muscal, E., Traipe, E., de Guzman, M.M. et al. MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus. Pediatr Radiol 40, 1241–1245 (2010). https://doi.org/10.1007/s00247-009-1540-y
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DOI: https://doi.org/10.1007/s00247-009-1540-y