Fulminant neuropsychiatric lupus leading to brain death: a case report

Neuropsychiatric systemic lupus erythematosus (NPSLE) represents a severe and heterogeneous manifestation of systemic lupus erythematosus (SLE), occasionally progressing to catastrophic outcomes. Although rare, fulminant forms with rapid neurological decline and brain death are exceptionally uncommon.

We report a 21-year-old woman with an 8-year history of SLE (articular, cutaneous, renal, and hematologic involvement) and hypothyroidism, who presented with acute neuropsychiatric symptoms and rapidly deteriorated to coma and brain death in less than 72 hours. Initial computed tomography (CT) was unremarkable. Laboratory results showed leukocytosis, negative anti-double-stranded DNA (dsDNA) antibodies, and normal complement (C3 and C4). Cerebrospinal fluid (CSF) revealed mild pleocytosis without infection. Despite intravenous methylprednisolone and multiple anticonvulsants, she developed refractory status epilepticus. Cranial CT angiography demonstrated severe cerebral and cerebellar edema, absent posterior circulation, and partial anterior circulation opacification; no definite hemorrhage was observed. Electroencephalogram (EEG) showed an isoelectric tracing after 24 hours off sedation, and brain death was confirmed in June 2025.

The clinical course was consistent with fulminant cerebral involvement, possibly related to lupus vasculitis, though histologic confirmation was not possible. This case highlights that NPSLE may progress with extreme rapidity, underlining the importance of early suspicion, aggressive immunosuppression, and advanced imaging techniques, such as vessel-wall magnetic resonance imaging (MRI), which may improve timely diagnosis and outcomes.