可逆性脳梁膨大部病変を有する軽症脳炎脳症（MERS）を合併した川崎病の1例

抄録

We present a case of Kawasaki disease (KD) complicated by clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). A 2-year-old boy was brought to the emergency outpatient unit because of recurrent convulsions. He had experienced two episodes of KD at the age of 1 year. Both episodes had been treated with high dose intravenous immunoglobulin therapy (IVIG; 2 g/kg/day) and the patient had recovered from both episodes without any sequelae. He was diagnosed as having KD based on the presence of 5 out of 6 criteria. After hospitalization, the patient exhibited a persistent disturbance of consciousness and was diagnosed as having MERS based on the presence of enhanced signals in the splenium of the corpus callosum on the 3rd day of illness. Initial treatment with IVIG plus pulsed methylprednisolone (30 mg/kg/day×3 days) for MERS was started. However, a high fever recurred on the 7th day. Additional treatment with IVIG plus intravenous prednisolone (2 mg/kg/day) was started on the 8th day. The patient's body temperature normalized on the 9th day and maintenance therapy with prednisolone was continued until the 28th day. Despite some risk factors for coronary artery lesion, the patient was discharged without any sequelae because of twice IVIG and aggressive steroid therapy including steroid pulse therapy.