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

Plexopathy

Disorder of brachial or lumbosacral plexus producing muscle weakness, sensory deficit, and/or reflex change not corresponding to the territory of single root or nerve.

Diagnostic criteria:

All of the following:

1. Characteristic signs and symptoms
   1. Brachial plexus: deep pain in shoulder, muscle weakness, sensory deficit and/or reflex impairment of arm
      or
   2. Lumbosacral plexus: deep boring pain in thigh, muscle weakness, sensory deficit and/or reflex impairment of leg
2. Positive EMG finding (concentric needle examination) and/or nerve conduction studies for EMG: >1 root or nerve abnormalities with sparing of paraspinal muscles for NCS: absent or reduced amplitude on motor or sensory nerve conduction
3. Normal MRI or CT scan (optional: myelogram) to rule out a higher neurologic lesion

Exclusions:

• Damage from injury, compression, tumor, aneurysm, radiation
• Cervical rib, thoracic outlet syndrome
• Plexus neuritis
• Toxic: heroin
• Infectious: Lyme disease, leprosy, herpes zoster

Associations:

• Diabetes mellitus
• Polyarteritis nodosa or other vasculitis
• Sarcoid

Ascertainment:

• History and physical examination
• EMG, NCS
• MRI or CT scan (optional: myelogram)

Record:

• Basic descriptors
• Location (brachial vs. lumbosacral)

(References: 112-113)

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