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

Headache

Discomfort in the region of the cranial vault.

I. Migraine

Migraine without aura: Idiopathic, recurrent headache manifested by attacks lasting 4-72 hours. Typical characteristics are unilateral location, pulsating quality, moderate to severe intensity, aggravation by routine physical activity, and associated with nausea, vomiting, photo- and phonophobia. At least 5 attacks fulfilling the above criteria.

Migraine with aura: Idiopathic, recurrent disorder manifested by attacks of neurologic symptoms localizable to cerebral cortex or brain stem, usually gradually developing over 5-20 minutes and lasting less than 60 minutes. Headache, nausea, and/or photophobia usually follow neurologic aura symptoms directly or after an interval of less than 1 hour. Headache usually lasts 4-72 hours, but may be completely absent.

II. Tension headache (episodic tension type headache)

Recurrent episodes of headaches lasting minutes to days. Pain typically pressing/tightening in quality, of mild to moderate intensity, bilateral in location, and does not worsen with routine physical activity. Nausea is rare, but photophobia and phonophobia may be present. At least 10 previous headaches fulfilling these criteria.

III. Cluster headache

Attacks of severe, strictly unilateral pain, orbital, supraorbital, and/or temporal, usually lasting 15-180 minutes and occurring from at least once every other day up to 8 times per day. Associated with one or more of the following: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, myosis, ptosis, eyelid edema. Attacks occur in series for weeks or months ("cluster" periods) separated by remissions of usually months or years.

IV. Headache from intracranial hypertension (Pseudotumor cerebri, benign intracranial hypertension)

All of the following:

1. Increased intracranial pressure (200 mm H₂O) measured by lumbar puncture
2. Normal neurologic findings except for papilledema and possible nerve VI palsy
3. No mass lesion and no ventricular enlargement on neuroimaging
4. Normal or low protein and normal white cell count in CSF
5. No evidence of venous sinus thrombosis

V. Intractable headache, nonspecific

Exclusions:

• Aseptic meningitis (including drug-induced)
• Drug-induced pseudotumor cerebri (oral contraceptives, sulfonamides, trimethoprim, etc.)
• CNS infection
• Tumors and other structural lesions
• Low intracranial pressure
• Trauma
• Metabolic headache that remits with elimination of cause (carbon monoxide exposure)
• Withdrawal (caffeine, etc.)
• Seizure/postictal state
• Sepsis
• Intracranial hemorrhage or vascular occlusion

Associations:

• Cranial neuropathies
• Headache associated with abnormalities of the eye, ear, sinus, teeth, temporomandibular joint, or cervical spine

Ascertainment:

• History (duration, localization, photophobia, visual disturbance, weakness, numbness, tingling, response to analgesia, aura)
• Physical examination including fundoscopy
• Lumbar puncture when indicated
• Imaging when indicated
• Antiphospholipid antibodies when indicated

Record:

• Basic descriptors
• Type of headache

(References: 20, 81-89)

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