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

Cerebrovascular Disease

Neurologic deficits due to arterial insufficiency or occlusion, venous occlusive disease, or hemorrhage. These are mainly focal deficits but may be multifocal in recurrent disease.

Diagnostic criteria:

One of the following and supporting radioimaging study:

1. Stroke syndrome: acute focal neurologic deficit persisting more than 24 hours (or lasting less than 24 hours with CT or MRI abnormality consistent with physical findings/symptoms
2. Transient ischemic attack: acute, focal neurologic deficit with clinical resolution within 24 hours (without corresponding lesion on CT or MRI)
3. Chronic multifocal disease: recurrent or progressive neurologic deterioration attributable to cerebrovascular disease
4. Subarachnoid and intracranial hemorrhage: bleeding documented by CSF findings MRI/CT
5. Sinus thrombosis: Acute, focal neurologic deficit in the presence of increased intracranial pressure

NB: The finding of unidentified bright objects on MRI without clinical manifestations is not classified at the present time.

Exclusions:

• Infection with space occupying lesions in the brain
• Intracranial tumor
• Trauma
• Vascular malformation
• Hypoglycemia

Associations:

• Diabetes mellitus
• Dyslipidemia
• Atherosclerotic vascular disease
• Atrial fibrillation
• Valvular heart disease
• Atrial septal defect
• Hypercoagulability state
• Antiphospholipid antibody syndrome
• Hypertension
• Smoking
• Cocaine or amphetamine abuse

Ascertainment:

• History and physical examination
• CT or MRI
• Angiogram as indicated
• Antiphospholipid antibodies
• Lumbar puncture as indicated
• EKG for suspected cardiac arrhythmias
• Echocardiography for suspected Libman-Sacks, valvular disease, or atrial septal defect
• Duplex ultrasound of carotid arteries for suspected carotid artery disease

Record:

• Basic descriptors
• Type of cerebrovascular pathology (infarct, hemorrhage, etc.)
• Antiphospholipid antibodies
• Other coagulation studies obtained

(References: 50-53)

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