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

Myelopathy

Disorder of the spinal cord characterized by rapidly evolving paraparesis and/or sensory loss, with a demonstrable motor and/or sensory cord level (may be transverse) and/or sphincter involvement.

Diagnostic criteria:

Usually rapid onset (hours or days) of one or more of the following

1. Bilateral weakness of legs with or without arms (paraplegia/quadriplegia); may be asymmetric
2. Sensory impairment with cord level similar to that of motor weakness;
   with or without bowel and bladder dysfunction.

Exclusions:

• Mass lesion causing compression of or within spinal cord (e.g., prolapsed disc, tumor, hematoma, or ruptured spinal arteriovenous malformation).
• Cauda equina lesion

Associations:

• Preexisting demyelinating syndrome
• Infections: herpes zoster, HIV
• Antiphospholipid antibodies

Ascertainment:

• History and physical examination:
• MRI or CT, myelogram as emergency
• CSF examination: cell count, protein, culture , oligoclonal bands, IgG index, cytology

Record:

• Basic descriptors
• Clinical features
• MRI/CT findings

(References: 102-105)

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