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

Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barré Syndrome)

Acute, inflammatory, and demyelinating syndrome of spinal roots, peripheral, and occasionally cranial nerves.

Diagnostic criteria:

A. Clinical features

1. Progressive polyradiculoneuropathy, usually ascending and predominantly motor, which peaks usually within 21 days or less
2. Reflex loss
3. Symmetric, may involve the trunk and may cause respiratory failure.

B. CSF

Increased CSF protein without pleocytosis

C. Supportive evidence by nerve conduction study (NCS) including F-wave ascertainment whereby there is ³ 1 abnormality in ³ 3 nerves (1)

The abnormalities are:

1. Conduction block in which the amplitude of compound muscle action potential diminishes with more proximal sites of nerve stimulation
2. F waves may be absent or prolonged
3. Slowing of conduction velocity
4. Prolongation of distal latencies

NB: Nerve conduction abnormalities may be subtle in early stages and may need to be repeated.

Exclusions:

• Acute spinal cord disease
• Botulism
• Poliomyelitis and other infections
• Acute myasthenia gravis

Ascertainment:

By history, physical examination, electrophysiologic study, and CSF examination

Record:

Basic descriptors with these modifications

• Onset (acute or insidious)
• Course (acute - days/subacute - weeks/chronic - months to years)

(References: 24, 27, 121-123)

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