Neuropsychiatric lupus (NPSLE)

Posted by Elaine Moore Jun 8, 2007

Neurospsychiatric systemic lupus erythematosus (NPSLE) refers to the neuropsychiatric symptoms that occur in patients with systemic lupus erythematosus (SLE). Although early studies showed that up to two-thirds of patients with systemic lupus erythematosus were at risk for developing neuropsychiatric symptoms, results of a five-year longitudinal study indicate that neuropsychiatric symptoms in systemic lupus erythematosus are not as common as previously described. Results of this study also show that in most SLE patients, cognitive dysfunction is not cumulative over time. This parallels the improved outcome for patients with systemic lupus erythematosus seen in the last decade. However, most current resources state that an average of 38 percent (range 14-75 percent) of patients with systemic lupus erythematosus may develop neuropsychiatric symptoms.

Neuropsychiatric Symptoms

Neuropsychiatric lupus is defined as a significant and unequivocal change in baseline neurologic and psychiatric function identified by history and physical examination. NPSLE may include one symptom or several different symptoms. About half of all patients with NPSLE only develop one neuropsychiatric symptom.

The milder symptoms such as focal/motor sensory deficits and headache are most commonly seen, while severe symptoms are rarely seen. Similar to other symptoms in systemic lupus, neuropsychiatric symptoms tend to wax and wane with alternating periods of flares and remissions. Neuropsychiatric symptoms in SLE include:

• Focal/motor sensory deficits including gait disturbances (most common symptom)
• Depression
• Poor judgment
• Seizures
• Headache
• Movement disorders
• Delirium
• Cranial and peripheral neuropathies
• Stroke
• Organic brain syndromes
• Memory impairment
• Cognitive dysfunction
• Pseudopapilloedema
• Dementia
• Psychosis

Who is Affected?

Only rarely do neuropsychiatric changes develop before a formal diagnosis of systemic lupus erythematosus is made. In most patients symptoms of NPSLE develop within the first two years of the onset of SLE although symptoms can occur at any time.

Possible Causes

Possible causes include antineuronal autoantibodies, vasculitis, cerebral hemorrhage, and secondary effects related to organ system lesions, such as kidney disease with hypertension, uremia, complications of therapy or superficial infections.

Immune System Changes

Antineuronal antibodies are seen in high titers in the cerebrospinal fluid in patients with NPSLE. Levels of interferon-alpha and interleukin-2 are also higher in the cerebrospinal fluid of patients with NPSLE, and over-expression of the inflammatory cytokines is commonly seen.

Antibodies directed against ribosomal P protein are also seen in nearly all patients with NPSLE compared to 10-20 percent of patients with SLE without organic brain involvement. Levels of antiphospholipid antibodies are also more likely to be seen in patients with NPSLE although their role in causing neuropsychiatric problems has not been established.

Resources:

Elaine Moore, Autoimmune Diseases and Their Environmental Triggers, Jefferson, NC: McFarland Publishing, 2002.

K. Waterloo, R. Omdal, G. Husby, and S. Mellgren, Neuropsychological Function in Systemic Lupus Erythtematosus: A Five-Year Longitudinal Study, Rheumatology 2002; 41: 411-415