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

Demyelinating Syndrome

Acute or relapsing demyelinating encephalomyelitis with evidence of discrete neurologic lesions distributed in place and time.

Diagnostic criteria:

Two or more of the following, each occurring at different times,

or one of the following occurring on at least two different occasions

1. Multiple discrete areas of damage to white matter within central nervous system, causing 1 or more limbs to become weak with sensory loss
2. Transverse myelopathy*
3. Optic neuropathy*
4. Diplopia due to isolated nerve palsies or internuclear ophthalmoplegia
5. Brain stem disease with vertigo, vomiting, ataxia, dysarthria, or dysphagia
6. Other cranial nerve palsies*

Exclusions:

• Infections, e.g., TB, HTLV-I, HIV, CMV, Borrelia, CNS Whipple’s disease, progressive multifocal leukoencephalopathy, syphilis
• Vitamin B₁₂ deficiency

Associations:

• Structural lesions, e.g., tumor, arteriovenous malformation
• Familial disorders, e.g., hereditary spastic paraplegia, ataxia, and leukodystrophies
• Sarcoid, Behçet’s disease, other vasculitis
• Multiple sclerosis

Ascertainment:

• History and physical examination
• CSF examination: cell count, protein, oligoclonal bands, IgG index, culture, cytology
• MRI
• Evoked potentials (optional)

Record:

• Basic descriptors

*These are also listed as separate case definitions as they can occur as isolated entities. Patients who meet criteria for these and for demyelinating syndrome should be classified as having both.

(References: 90-98)

To Index | Previous (Cognitive Dysfunction) | Next (Headache) | To References