In a 72-year-old female, subacute right hemiplegia and aphasia appeared in late May 2011. The results of hematology, a cerebrospinal fluid test, (13)F-FDG-PET, and cephalic MRI suggested intravascular/malignant lymphoma. Brain biopsy was performed. Pathological findings did not suggest a malignant tumor. In the perivascular space, the infiltration of neutrophils or histiocytes was observed. The patient was referred to the Department of Neurology. Based on the results of various examinations, infection was ruled out, and steroid therapy was conducted. Marked improvement was achieved. Subsequently, the results of human leukocyte antigen (HLA) typing showed B54/Cw1. As dermal findings were absent, it was impossible to make a definitive diagnosis of neuro-Sweet disease, but the disorder was regarded as a neuro-neutrophilic disease, which is a more comprehensive entity. Few studies have reported brain tissue findings of active neuro-neutrophilic disease. We report the present case, which will contribute to future research.
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