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Neuropsychiatric manifestations of systemic lupus erythematosus

Peter H Schur, MD
Section Editors
Michael J Aminoff, MD, DSc
Jonathan M Silver, MD
Deputy Editors
Janet L Wilterdink, MD
David Solomon, MD


Central nervous system neuropsychiatric lupus refers to the various psychiatric and neurologic manifestations that develop secondary to involvement of the CNS in patients with systemic lupus erythematosus (SLE). These clinical features are characterized by some investigators as either diffuse (eg, encephalopathy, coma, depression, and psychosis) or complex (eg, encephalopathy with stroke or seizure, and psychiatric presentation with stroke or seizure) [1]. Disturbances of mental function are the most common symptom.

Because of the varied diagnostic criteria associated with these manifestations, the American College of Rheumatology (ACR) has formulated case definitions, reporting standards, and diagnostic testing recommendations for the 19 neuropsychiatric SLE syndromes (table 1) [2]. The full definitions can be found at the following Internet address: www.rheumatology.org/publications/ar/1999/aprilappendix.asp.

A psychiatric disturbance due to CNS lupus is a diagnosis of exclusion; all other possible causes of the observed symptoms must therefore be considered, including infection, electrolyte abnormalities, renal failure, drug effects, mass lesions, arterial emboli, and primary psychiatric disorders (such as bipolar disorder or severe stress disorder resulting from a chronic and life-threatening disease) [3]. One clue to the diagnosis is that most acute psychiatric episodes occur during the first two years after the onset of SLE [4,5].

The term lupus cerebritis refers to the neuropsychiatric manifestations of lupus that appear to have an organic basis, rather than a specific pathophysiologic mechanism. The distinction between organic and functional causes of some neuropsychiatric symptoms can occasionally be made by assaying for specific autoantibodies.

Some investigators believe that a strong association exists between the occurrence of neuropsychiatric symptoms and the presence of antineuronal antibodies and other antibodies [6-9].

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Literature review current through: Nov 2017. | This topic last updated: Nov 18, 2014.
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