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

Polyneuropathy

Acute or chronic disorder of sensory and motor peripheral nerves with variable tempo characterized by symmetry of symptoms and physical findings in a distal distribution.

Diagnostic criteria

One or both of the following:

A. Clinical manifestations

1. Clinical demonstration of distal sensory and/or motor deficit
2. Symmetry of signs/symptoms, and/or

B. Confirmation by EMG

1. Concentric needle examination demonstrating denervation of muscle, or
2. Nerve conduction study demonstrating axonal or demyelinating neuropathy

Exclusions:

• Vitamin deficiencies: B₁₂, niacin, thiamine
• Hypothyroidism

Associations:

• Heavy metal and solvent exposures: arsenic, lead, mercury, n-hexane, etc.
• Drug toxicity: isoniazid, vincristine, phenytoin, colchicine, etc.
• Leprosy, HIV, diphtheria, Lyme disease
• Diabetes, uremia, amyloid, alcoholism, porphyria, etc.
• Paraproteinemia, cryoglobulinemia
• Sjö gren’s syndrome
• Inherited forms: Charcot-Marie-Tooth, Fabry’s disease, Tangier’s disease, familial amyloid polyneuropathy, etc.

Ascertainment:

By history and examination and/or electrophysiologic studies

Record:

• Basic descriptors
• Predominant clinical feature (pure motor, pure sensory, motor-sensory or sensory-motor)

(References 114, 115)

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