What's on this Page
Neurologic manifestations of systemic lupus erythematosus
What are the neurologic manifestations of systemic lupus erythematosus (SLE)?
Neurologic symptoms and signs appear as manifestations of SLE in 30% to 40% of patients, with 40% to 50% of these manifestations occurring within 1 to 2 years of diagnosis.
Major risk factors for neurologic manifestations of SLE include generalized disease activity with treatment with cytotoxic agents or steroids, previous neurologic manifestations including stroke and seizure, and positive antiphospholipid antibodies.
Central dysfunction includes neuropsychiatric and behavioral changes, such as dementia, psychosis, and confusional states (the most common central manifestation of SLE).
While severe cognitive dysfunction occurs in only 2% to 5% of cases, mild to moderate cognitive dysfunction is common. Localizing neurologic findings include hemiparesis, chorea, tremor, cerebellar ataxia, cranial neuropathies including optic neuritis, and transverse myelitis.
Aseptic meningitis, seizures, and signs of increased intracranial pressure may also develop in SLE patients.
The mean 5-year survival for patients with neurologic symptoms is 30% less than that of SLE patients without neurologic problems. Vasculitis with CNS hemorrhage accounts for a large portion of this difference.
SLE may result in a vasculitic peripheral neuropathy that may manifest as mononeuropathy, mononeuritis multiplex, a symmetric distal sensorimotor deficit. Myositis occurs in 25% of patients with SLE but is a serious complication only when the myocardium is involved.
Sources
Agrawal L, Habib Z, Emanuele NV: Neurologic disorders of mineral metabolism and parathyroid disease. Handb Clin Neurol 120:737-748, 2014. Riggs J: Neurologic complications of electrolyte disturbances.
In Aminoff MJ, Josephson SA (eds): Aminoff’s neurology and general medicine , Academic Press, 2014. Boumpas DT, Austin HA 3rd, Fessler BJ, et al: Systemic lupus erythematosus: emerging concepts. Ann Intern Med 122:940-950, 1995. Bertsias GK, Boumpas DT: Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol 6:358-367, 2010.
