Publication:

Neuropsychiatric systemic lupus erythematosus with normal CSF and EEG: a diagnostic challenge in the setting of antiphospholipid syndrome and vasculitis

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a diagnostically challenging manifestation of SLE. We report a 56-year-old woman with known SLE, antiphospholipid syndrome, vasculitis, and seizure disorder who presented with refractory seizures, hallucinations, and altered sensorium. Despite entirely normal cerebrospinal fluid (CSF) analysis and electroencephalography (EEG), MRI brain revealed an old microhemorrhage in the right thalamocapsular region with diffuse cerebral atrophy, and anti-dsDNA-NcX IgG was positive. A diagnosis of NPSLE was established on this basis. She was treated with intravenous immunoglobulin, intravenous dexamethasone, intravenous cyclophosphamide, and antiepileptic agents, achieving sustained clinical remission at three-month follow-up. This case highlights that normal CSF and EEG do not exclude NPSLE, and that serological and neuroimaging findings must be interpreted together to reach a timely diagnosis and initiate appropriate immunotherapy.