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Appendix A: Case Definitions for Neuropsychiatric Syndromes in Systemic Lupus Erythematosus

Aseptic Meningitis

Syndrome of fever, headache, and meningeal irritation with CSF pleocytosis, and negative CSF cultures.

Diagnostic criteria:

All the following:

  1. Acute or subacute onset of headache with photophobia, neck stiffness, and fever
  2. Signs of meningeal irritation
  3. Abnormal CSF

Exclusions:

CNS or meningeal inflammation due to:

  1. Infection by bacteria, mycobacteria, viruses, fungi, parasites
  2. Subarachnoid hemorrhage
  3. Malignancy (leukemia, lymphoma, or carcinoma) or granulomatous disease (sarcoidosis)
  4. Medications: NSAIDs, intravenous immunoglobulin, azathioprine, etc.

Ascertainment:

History and physical examination

CSF examination for cells, glucose, protein, culture

Record:

  • Basic descriptors

(References: 104, 105)

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