Brain MRI Revealing the Probable Pathophysiology of Neuropsychiatric Lupus: A Case Report

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease with a wide array of manifestations in different organ systems. Involvement of the central nervous system (CNS), also known as neuropsychiatric SLE (NPSLE), often presents with seizures, mental status changes, focal neurological findings, and cognitive impairment. While many underlying pathophysiologic mechanisms have been described, no unifying etiology has been determined for NPSLE, thus complicating treatment.  We highlight the case of a 41-year-old female with SLE who presented to the emergency department after several generalized tonic-clonic seizure episodes. On physical examination, she was oriented only to self, had poor speech production, and responded only to noxious stimuli. Electroencephalography (EEG) showed right temporal focal slowing followed by a right temporal focal onset seizure. The patient was started on levetiracetam, lacosamide, and valproic acid. Pertinent lab results showed active systemic lupus. Her brain MRI revealed relatively symmetric bilateral medial temporal fluid-attenuated inversion recovery (FLAIR) hyperintensities compatible with limbic encephalitis. Her neurological condition and seizures persisted despite treatment with multiple anticonvulsants and oral prednisone. Once intravenous immunoglobulin (IVIg) was initiated, rapid symptom relief was achieved within 48 hours in refractory NPSLE with limbic encephalitis features, highlighting its efficacy when anticonvulsants (levetiracetam, lacosamide, valproic acid) and steroids (prednisone 60 mg) fail. This case demonstrates the importance of understanding the various underlying pathophysiologies of NPSLE to better target clinical management. The use of modern neuroimaging MRI techniques may aid in this pursuit.