ABSTRACT
BACKGROUND: The diagnosis of neuropsychiatric lupus erythematosus (NPSLE) can be difficult and has to be differentiated from neurologic complications that result from hypertension, drugs, infection, uremia, and metabolic changes. DIAGNOSTICS: There is no single test which is diagnostic. Therefore, the clinical presentation, serologic tests and neuroimaging techniques have to be combined to support the diagnosis of cerebral lupus. Magnetic resonance imaging (MRI) is routinely used with a sensitivity of 50-87%. However, the abnormalities such as white matter lesions or brain atrophy are nonspecific and were also found in asymptomatic patients (16-52%). A negative MRI result does not exclude a diagnosis of cerebral lupus. Antibodies against phospholipids are an important immunoserologic marker due to the close association with thromboembolic events. Echocardiography and cerebrospinal fluid examination should be added to rule out cardiac embolic disease and CNS infection. Functional brain imaging techniques such as single-photon emission computed tomography, positron emission tomography, magnetization transfer imaging or magnetic resonance spectroscopy may be helpful especially in patients with unconspicuous MRI, but the findings are not SLE-specific. THERAPY: The treatment of cerebral lupus is empiric, due to a lack of randomized studies. Inflammatory brain lesions are treated with corticosteroids and immunosuppressive drugs (e. g., cyclophosphamide). Anticoagulant therapy with coumarins (at a target INR of 3.0-3.5) is recommended in cases of thrombotic events associated with antiphospholipid antibodies. However, no studies exist on patients with arterial thrombosis, including strokes, supporting this target INR.
