Systemic lupus erythematosus is a chronic autoimmune inflammatory disorder caused by abnormal deposition of antigen-antibody complexes. Kaposi is credited with the first description of the disorder in 1872 and noted "disturbed neurologic function" in his report (92). Although any organ system in the body can be affected, patients most often have arthritis (88%), butterfly rash (79%), pericarditis (64%), and kidney dysfunction (48%). Neurologic sequelae of lupus are common and have been estimated at 37% of patients (93). Neurologic manifestations are quite diverse and can include motor deficits, seizures, cranial nerve dysfunction, headaches, cerebral hemorrhages, and myelitis. Cranial neuropathy has been estimated in 11% of cases (93). The facial nerve is the most common cranial nerve involved, with a roughly 5% prevalence. Bilateral facial involvement has also been described (94).
The underlying pathogenesis of neurologic involvement is unknown, but many autopsy studies have implicated microvascular injury as the mechanism. Some investigators have noted deposition of antibody-antigen complex within the choroid plexus with ensuing damage and dysfunction of CSF production. The facial nerve, in particular, appears to be vulnerable, with pathologic lesions demonstrated along its course from the facial nucleus to the neuromuscular junction (92).
Diagnosis is confirmed with specific blood tests including elevated ESR, antinuclear antibodies (ANA), anti-Sm, anti-DNA, anti-ribonuclear protein (anti-RNP), anti-Ro (SSA), anti-La (SSB), and anticardiolipin antibody. A urinalysis commonly identifies elevated protein consistent with nephrotic syndrome characteristic of the kidney damage caused by the antigen-antibody deposition in lupus.
Treatment of lupus involves immunosuppressive therapy, usually high doses of corticosteroids for exacerbations. Immuran and cytoxan have also been used. Nonsteroidal anti-inflammatory drugs can be used for mild symptom control. Antimalarials such as hydroxyquinine have been shown to reduce the severity and frequency of exacerbations. Plasmapheresis, monoclonal antibodies, and total lymphoid irradiation have also shown some benefit but are considered experimental. The prognosis is variable, with 84% of patients demonstrating partial or complete improvement in their neurologic dysfunction with corticosteroid treatment (93).
See Chapter 1 for detailed discussion of systemic lupus emythemetosus (SLE).
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