Neuropsychiatric Systemic Lupus Erythematosus with Mixed Focal and Diffuse Involvement during an Acute Flare: A Case Report

Abstract

Neuropsychiatric manifestations occur in a minority of systemic lupus erythematosus (SLE) patients and can present with a diverse spectrum of symptoms, complicating the diagnosis and treatment. This report discusses a case involving a 58-year-old female patient who experienced an acute flare of systemic lupus erythematosus with multisystem involvement. Initial treatment with methylprednisolone did not alleviate her neurological symptoms. Antiphospholipid antibody syndrome was ruled out, and neuroimaging studies were normal. After exclusion of alternative etiologies, the patient was diagnosed with NPSLE. The patient had a rare combination of neuropsychiatric systemic lupus erythematosus, presenting with both focal (peripheral neuropathy) and diffuse (cognitive and behavioral changes) symptoms. The patient's neurological symptoms improved significantly with cyclophosphamide treatment, and she remains in remission on Mycophenolate mofetil maintenance therapy. This case underscores the complexity of neuropsychiatric systemic lupus erythematosus and the need for thorough evaluation. Additionally, it highlights the potential effectiveness of cyclophosphamide in managing refractory neuropsychiatric symptoms in systemic lupus erythematosus patients.