Diagnostic approach to the neuropsychiatric manifestations of systemic lupus erythematosus
- Peter H Schur, MD
Peter H Schur, MD
- Editor-in-Chief — Rheumatology
- Section Editor — Basic Science
- Professor of Medicine
- Harvard Medical School
- Section Editors
- Michael J Aminoff, MD, DSc
Michael J Aminoff, MD, DSc
- Editor-in-Chief — Neurology
- Section Editor — Medical Neurology
- Professor of Neurology
- University of California, San Francisco School of Medicine
- Jonathan M Silver, MD
Jonathan M Silver, MD
- Section Editor — Mental and Medical Disorders
- Clinical Professor of Psychiatry
- New York University School of Medicine
Systemic lupus erythematosus (SLE) may involve the nervous system and present with a number of different neurologic and psychiatric syndromes (table 1). These neuropsychiatric manifestations may mimic symptoms due to intercurrent illness, medication use, and functional disturbances (table 2). The term lupus cerebritis refers to the neuropsychiatric manifestations of lupus that appear to have an organic rather than psychiatric basis, rather than a specific pathophysiologic mechanism.
Although it would be desirable to have diagnostic tests that establish a specific diagnosis of neuropsychiatric lupus, such tests do not exist. Thus, the approach to patients with neuropsychiatric symptoms consists of studies that establish the diagnosis of SLE, distinguish between organic and functional etiologies, and exclude symptoms not due to SLE. A typical workup for the patient with acutely altered behavior and mental status is outlined in Table 3 (table 3).
Diagnostic testing, and the interpretation of such testing, in patients suspected of having neuropsychiatric manifestations of SLE are reviewed here. Details of the clinical neurologic and psychiatric manifestations of SLE are presented separately. (See "Neurologic manifestations of systemic lupus erythematosus" and "Neuropsychiatric manifestations of systemic lupus erythematosus".)
Serologic tests are typically used to help establish the diagnosis of SLE. The antinuclear antibody (ANA) titer is positive in virtually all patients with this disorder. However, the presence of ANA is not a very specific finding, and many non-SLE patients have symptoms such as headache or fatigue with only a weakly positive ANA. Serum levels of anti-DNA and anti-Sm antibodies and low levels of complement are useful in this setting to corroborate the diagnosis of SLE. (See "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults".)
In the patient with established SLE, an association between neuropsychiatric symptoms and specific antibodies has been demonstrated (by some) in a few circumstances [1-3]:
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