Cerebrovascular Accident as a First Manifestation of Neuropsychiatric Systemic Lupus Erythematosus; a Comprehensive Review; a Case Report

Systemic lupus erythematosus (SLE) is an autoimmune connective-tissue disease that mostly affects women of childbearing age. One of the important symptoms of lupus erythematosus is neuropsychiatric (NP) manifestations that mostly present with neurologic syndromes as well as confusion, lethargy, mood changes, and even psychosis. The case presented in this article is a 38-year-old woman who had progressive left side hemiparesis and cognitive dysfunction. According to the brain MRI and further examinations, she had a cerebrovascular accident (CVA) along with Systemic lupus erythematosus. Due to the definite SLE, the positivity of antiphospholipid antibodies, and the occurrence of CVA, the patient was diagnosed as definite neuropsychiatric systemic lupus erythematosus (NPSLE). NPSLE is a general explanation mentioning a series of neurological and psychiatric features directly related to systemic lupus erythematosus. Due to the scarcity of clinical trials in NPSLE, we obtain most of the data from case series and case reports. Two different mechanisms are recognized to associate to NPSLE: (1) Inflammatory and autoimmune-mediated events (2) Thrombotic and ischemic associated with microangiopathy, and hemorrhage. In the acute phase, management of patients with NPSLE diagnosis does not vary from other non-SLE neurologic subjects which present with the neurological manifestation. High-dose glucocorticoids and intravenous cyclophosphamide or even rituximab are used in patients with inflammatory and autoimmune pathophysiology. Anticoagulation and antiplatelet agents are used in treatment when the patient has vascular thrombosis. Antimalarial medications such as hydroxychloroquine may play a protective role against the worsening of brain lesions. This article supplies a comprehensive review of the literature on the pathophysiology, epidemiology, diagnostic approach, and management of NPSLE. We also explain the most common pharmacological treatments for NPSLE, based on a search of articles as well as a complete mention of an NPSLE case report.

Keywords:Cerebrovascular accident; Neuropsychiatric systemic lupus erythematosus; SLE; NPSLE

Abbreviations:NPSLE: Neuropsychiatric Systemic Lupus Erythematosus; SLE: Systemic Lupus Erythematosus; CVA: Cerebrovascular Accident; NP: Neuropsychiatric; MRI Magnetic Resonance Imaging; MRA: Magnetic Resonance Angiography; MRS: Magnetic Resonance Spectroscopy; APS: Antiphospholipid Syndrome; SS: Sjogren’s Syndrome; aPLs: Antiphospholipid antibodies