Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature

Zhang,Pingping; Li,Xiaofeng; Li,Yating; Wang,Jing; Zeng,Huasong; Zeng,Xiaofeng

doi:10.3892/br.2014.380

Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature

Authors:
- Pingping Zhang
- Xiaofeng Li
- Yating Li
- Jing Wang
- Huasong Zeng
- Xiaofeng Zeng
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Affiliations: Department of Pediatrics, Allergy, Immunology and Rheumatology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University and First Clinical Medical College, Jinan University, Guangzhou 510120, P.R. China, Department of Pediatrics, The Third Affiliated Hospital of Sun Yat‑Sen University, Guangzhou 510630, P.R. China, Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, P.R. China
Published online on: November 7, 2014 https://doi.org/10.3892/br.2014.380
Pages: 55-58

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Abstract

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5‑year‑old male with a previous diagnosis of systemic‑onset juvenile idiopathic arthritis (SoJIA) and macrophage‑activation syndrome who developed posterior reversible encephalopathy syndrome during treatment with glucocorticoids, disease‑modifying antirheumatic drugs and biological agent (etanercept) therapy. After ~5 days of treatment, the patient made a complete clinical recovery; the magnetic resonance imaging reviewed 2 weeks later showed that the previous hyper‑intensity signal had disappeared and the multiple lesions in the brain had been completely absorbed. The case report shows that, conforming to recent literature, SoJIA in infants should be considered a risk factor for developing RPLS. The clinical manifestations of the disease are multiple, but usually reversible, and the patients mostly have a good prognosis. Rapid diagnosis and treatment is essential as early treatment may prevent progression to irreversible brain damage. By increasing the awareness of RPLS, the patient care may improve and further insight may be gained.

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