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Essentials of Diagnosis

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  • According to the American College of Rheumatology, the following are central nervous system (CNS) manifestations of systemic lupus erythematosus (SLE):
    • Acute confusional state.
    • Anxiety disorder.
    • Aseptic meningitis.
    • Cerebrovascular disease.
    • Cognitive dysfunction.
    • Demyelinating disease.
    • Headache.
    • Mood disorder.
    • Movement disorder (chorea).
    • Myelopathy.
    • Psychosis.
    • Seizure disorder.

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General Considerations

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The general term “neuropsychiatric lupus” is being superseded by the understanding that lupus can affect the nervous system in diverse ways, each with distinctive clinical findings, pathogenesis, prognosis, and treatment. The American College of Rheumatology distinguished 12 central neurologic manifestations of SLE. Neurologists subdivide these in greater detail, noting, for example, many different mechanisms and localizations of strokes or seizures. Neurologic manifestations of lupus often occur early in the disease. The CNS syndromes discussed below are presented in approximate order of prevalence; however, prevalences are inexact due to variations of definition and clinical sampling. Patients with SLE are also at risk for peripheral nervous system disease (Table 73–1).

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Table Graphic Jump Location
Table 73–1. Peripheral Nervous System Manifestation of Systemic Lupus Erythematosus.
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Cognitive Dysfunction & Acute Confusional State

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Clinical Findings

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Cognitive dysfunction, especially in domains such as visual or verbal memory, attention, executive function, and psychomotor speed, is the most common neuropsychiatric aspect of lupus. These disturbances can range from subtle to severe. Prevalence figures vary greatly, depending on diagnostic criteria and extent of psychometric testing. Mild cognitive dysfunction typically waxes and wanes without clear correlation to other aspects of disease activity. Patients with antiphospholipid antibodies have increased risk of developing cognitive dysfunction.

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Severe cognitive dysfunction, including acute confusional states or delirium, affects only a small percentage of lupus patients and can overlap with other neuropsychiatric aspects of lupus, such as psychosis and affective disorders. Patients with these more severe neuropsychiatric presentations usually have serum and cerebrospinal fluid (CSF) antibodies against the NR2-NMDA receptor. These antibodies are probably not solely responsible for the mental changes because titers often remain elevated in the CSF months after symptoms have resolved. Moreover, these antibodies are less likely to be elevated in lupus patients with focal neurologic syndromes. Other autoantibodies, such as those directed against antiribosomal P, can occur in patients with neurologic manifestations of lupus, but none is particularly helpful in diagnosing whether or not a patient has lupus or if new neurologic findings are due to SLE or other etiology.

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In 50% or more of patient with lupus, brain MRI shows T2-bright lesions in the white matter. These lesions can be present in patients without clinical CNS disease. However, the lesions are more common among patients with ...

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