Neuro-Sweet's disease manifesting as limbic encephalitis and ventriculitis

A case of neuro-Sweet's disease (NSD) that exhibits rare and characteristic MRI findings mimicking limbic encephalitis and ventriculitis is presented. The patient showed consciousness disturbance and left hemiparesis but lacked cutaneous symptoms typical of NSD. The diagnosis was further supported by increased neutrophil count, elevated interleukin-6 levels in cerebrospinal fluid, positive results for HLA-B54 and HLA-Cw1 compatible with NSD, as well as neutrophilic inflammation confirmed in a brain biopsy specimen. Neurological impairment was remarkably improved following treatment with intravenous methylprednisolone pulse therapy as well as oral prednisolone and colchicine. NSD should be considered as a differential diagnosis in cases with neuroimaging findings suggestive of limbic encephalitis and ventriculitis.