In September 2000, a 22-year-old female was admitted to our hospital due to high grade
fever, liver
enzymes elevation and
pancytopenia. Bone marrow aspiration was performed, and hemophagocytosis was present. Epstein-Barr virus (EBV)
DNA was positive in her peripheral blood, and we diagnosed the case as EBV-associated
hemophagocytic syndrome (EB-VAHS) after excluding other
malignancies. The initial
therapy including
etoposide and
dexamethasone was started. As severe
leukocytopenia developed,
etoposide was stopped and
cyclosporin A (CsA) was administered continuously. Four days after administration of CsA, she developed convulsive
seizures with
loss of consciousness. An MRI demonstrated decreased signal with T1-weighting and high signal with T2-weighting in the subcortical white matter including the posterior lobe. We stopped CsA infusion, and
glycerol was administered. Soon the symptom disappeared. When patients developed an episode of convulsive seizure, other diagnostic possibilities were central nervous system (CNS) involvement of hemophagocytosis, EBV
encephalitis and
acute disseminated encephalomyelitis (ADEM). CsA neurotoxicity must be considered even in the case of EB-VAHS with administration of CsA. As previously reported, Fluid-attenuated Inversion Recovery (FLAIR) imaging improved diagnostic confidence and conspicuity of the T2 hyper intense lesions of CsA neurotoxicity, as well as
tacrolimus encephalopathy, typically in the subcortical white matter. Key words;
Cyclosporin neurotoxicity; Epstein-Barr virus associated-
Hemophagocytic syndrome; Magnetic Resonance Image (MRI).