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

Movement Disorder (Chorea)

Chorea: Irregular, involuntary and jerky movements, that may involve any portion of the body in random sequence. Each movement is brief and unpredictable.

Diagnostic criteria:

Both of the following:

1. Observed abnormal movements
2. Random, unpredictable sequence of movements

Exclusions:

• Wilson’s disease
• Huntington’s disease (and other hereditary disorders)
• Medications (neuroleptics, oral contraceptives, phenytoin, L-dopa, calcium channel blockers)
• Illicit drugs

Associations:

• Stroke, vascular malformation, hypoxic damage
• Tumor
• Pregnancy (chorea gravidarum)
• Rheumatic fever (Sydenham’s chorea)
• Antiphospholipid antibodies

Ascertainment:

• History and examination
• Antiphospholipid antibodies
• Serum copper, ceruloplasmin, slit lamp examination to exclude Wilson’s disease when clinically indicated
• MRI of the brain when clinically indicated

Record:

• Basic descriptors
• Description of abnormal movement

(References: 99-101)

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