Systemic lupus erythematosus (SLE) is an autoimmune disease frequently manifested by neuropsychiatric involvement, which occurs in up to 75% of patients, depending on the type of manifestations included. Primary involvement may vary from subtle signs, such as headache and mood disorders, to severe, life-threatening conditions, such as stroke, myelopathy, and acute confusional state. Any part of the peripheral or central nervous system (CNS) may be affected by the disease. The diagnosis of primary CNS involvement in SLE is often difficult because both focal and diffuse manifestations may occur. A wide range of differential diagnoses has to be considered, including metabolic abnormalities, infections, uremia, hypertension, and drug therapy.
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Central nervous system manifestation following American College of Rheumatology case definitions (adapted from American College of Rheumatology Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature14).
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