Abstract
A 17-year-old Japanese woman with refractory Epstein–Barr virus-associated hemophagocytic syndrome (EB-VAHS) developed meningoencephalopathy with seizures. A magnetic resonance imaging (MRI) examination showed multiple lesions with gadolinium enhancement in the cortex of the hemisphere, hypothalamus and brainstem. In addition, contrast-enhanced fluid-attenuated inversion recovery (FLAIR) images showed the abnormal enhancement of the leptomeninges. A cerebrospinal fluid analysis detected Epstein–Barr virus polymerase chain reaction positivity, the absence of pleocytosis and an increased protein level. Prompt initiation of high-dose steroid therapy dramatically improved the symptoms and abnormal MRI findings. This case suggests that contrast-enhanced FLAIR images and Epstein–Barr virus polymerase chain reaction using a cerebrospinal fluid sample might be useful for the early detection of central nervous system involvement in VAHS.
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