Adult-onset Still's disease with concurrent acute necrotizing encephalopathy: a case report

Acute necrotizing encephalopathy (ANE) is a rare encephalopathy characterized by multiple symmetrical brain lesions, mainly involving thalami. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition of unknown cause characterized by fever, sore throat, rash and joint pain. Both entities are considered to be triggered by infections and associated with hypercytokinemia.

A 46-year-old male was diagnosed with AOSD at local hospital because of 3-week-long high fever, sore throat, arthralgia, transient skin rash, lymphadenopathy, leukocytosis, hyperferritinemia, and absence of antinuclear antibodies (ANA) and rheumatoid factor (RF). Corticosteroids were not used because of delayed diagnosis. Three weeks after the onset, the patient suddenly fell unconscious and was transferred to our hospital. Brain CT and MRI revealed symmetrical lesions involving thalami, striatum and brain stem, consistent with ANE. One day after admission, his condition aggravated and brain CT revealed hemorrhage in the lesions. He died 3 days after admission.

We report a rare case of ANE preceded by AOSD. The underlying mechanism is still unclear. Early recognizing of the two conditions is difficult but prognostically important.