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

Neuropathy, Cranial

Disorder of sensory and/or motor function of a specific cranial nerve(s).

Diagnostic criteria:

Syndrome corresponding to specific nerve function:

1. Olfactory nerve: Loss of sense of smell, distortion of smell, and loss of olfactory discrimination
2. Optic nerve: Decrease or loss of visual acuity, diminished color perception, afferent pupillary defect and visual field deficits
3. Oculomotor nerve: Ptosis of the upper eyelid and inability to rotate eye upward, downward, or inward (complete lesion), and/or dilated nonreactive pupil and paralysis of accommodation (interruption of parasympathetic fibers only)
4. Trochlear nerve: Extorsion and weakness of downward movement of affected eye
5. Abducens nerve: Weakness of eye abduction
6. Trigeminal nerve: Paroxysm of pain in lips, gums, cheek, or chin initiated by stimuli in trigger zone (trigeminal neuralgia) and sensory loss of the face or weakness of jaw muscles
7. Facial nerve: Unilateral or bilateral paralysis or facial expression muscles, impairment of taste, and hyperacusis (painful sensitivity to sounds)
8. Vestibulo-cochlear nerve: Deafness, tinnitus (cochlear), dizziness and/or vertigo (vestibular)
9. Glossopharyngeal nerve: Swallowing difficulty, deviation of soft palate to normal side, anesthesia of posterior pharynx and/or glossopharyngeal neuralgia (unilateral stabbing pain in root of tongue and throat, triggered by coughing, sneezing, swallowing, and pressure on ear tragus)
10. Vagus nerve: Soft palate droop, loss of the gag reflex, hoarseness, nasal voice, and/or loss of sensation at external auditory meatus.
11. Accessory nerve: Weakness and atrophy of sternocleidomastoid muscle and upper part of trapezius muscle.
12. Hypoglossal nerve: Paralysis of one side of tongue with deviation to the affected side

Exclusions:

• Skull fracture
• Tumor: meningioma, carcinomatous meningitis, aneurysm
• Infection: herpes zoster, neuroborreliosis, syphilis, mucormycosis
• Miller Fisher syndrome

Associations:

• Nutritional: thiamine deficiency
• Metabolic: diabetes mellitus
• Inflammatory: multiple sclerosis
• Ischemic: giant cell arteritis, brainstem stroke
• Infiltrative: sarcoid

Ascertainment:

History and physical examination

Record:

• Basic descriptors

(References: 108-110)

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