Article in Japanese
A case of clinically mild encephalitis/encephalopathy with a reversible splenial lesion accompanied by Epstein-Barr virus hemophagocytic lymphohistiocytosis
Mitsuhiro MIYASHITA1), Masao TOGAWA1), Aya KITAGUCHI1), Kiyoko AMO1)
A previously healthy 3-year-old Japanese boy developed fever, with hepatosplenomegaly. A laboratory test revealed pancytopenia, hyperferritinemia, high soluble interleukin-2 receptor, and positive Epstein-Barr virus DNA, leading to the diagnosis of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH). While suffering from EBV-HLH, consciousness disorder and hyponatremia with vomiting appeared in the patient. Brain diffusion-weighted imaging acquired by magnetic resonance imaging (MRI) demonstrated clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). EBV-HLH is a severe disease caused by excessive cytokine production. Central nervous system involvement in EBV-HLH is associated generally with poor outcome. On the other hand, this case and previous reports suggest that MERS accompanied by EBV-HLH has a comparably good prognosis with no specific therapy. MERS onset based on hypercytokinemia may relate to increasing secretion of antidiuretic hormone and hyponatremia.
1) Department of Pediatric Emergency, Children's Medical Center, Osaka City General Hospital
Key words | clinically mild encephalitis/encephalopathy with a reversible splenial lesion, Epstein-Barr virus, hemophagocytic lymphohistiocytosis, hypercytokinemia, hyponatremia |
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Received | August 3, 2020 |
Accepted | January 25, 2021 |
33 (2):93─100,2021
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