Central nervous system lupus (CNS lupus) is a manifestation of systemic lupus erythematosus (SLE) that affects the brain and spinal cord, causing cognitive dysfunction, headaches, visual disturbances, and movement disorders. It can be caused by inflammation, vasculitis, antiphospholipid antibodies, or anti-neuronal antibodies. Treatment involves anti-inflammatory and immunosuppressant drugs, anticoagulants, and plasmapheresis.
Central nervous system lupus (CNS lupus) is one of the manifestations of the disease called systemic lupus erythematosus (SLE). It is also called neuropsychiatric lupus (NPSLE). In some SLE patients, nervous system disease is a major cause of loss of quality of life and death. CNS lupus often presents as a cognitive dysfunction, particularly affecting memory and reasoning. Any location in the brain and spinal cord can be involved, so presentations of this type of lupus can vary.
The primary symptom of CNS lupus is cognitive dysfunction. Affected patients often have difficulty remembering things or are unable to think or reason clearly. Other CNS lupus symptoms include headaches, peripheral neuropathies, visual disturbances, and movement disorders. Some may experience autonomic neuropathy, which presents as redness or mottling of the skin with no apparent environmental trigger. Others experience psychosis, aseptic or noninfectious meningitis, strokes, seizures, stupor, and coma.
Although the underlying pathogenesis of SLE is the production of autoantibodies against cells of the body, CNS lupus is caused by various mechanisms. It is proposed that inflammation or vasculitis of blood vessels due to the deposition of immune mediators in the vascular chain may cut off blood supply to the brain. Additionally, antiphospholipid antibodies can attack red blood cells, leading to the formation of clots or thrombi within blood vessels, and these clots also impair blood supply to the brain. Some lupus patients have anti-neuronal antibodies that attack neurons. Inflammatory mediators such as interleukin-1, interleukin-6, and interferon-gamma can be elevated, leading to inflammation that interferes with neural function.
Hormonal dysfunction, treatments for SLE, and infections due to suppressed immune system can also contribute to CNS dysfunction. Previously, CNS lupus was thought to affect only 25% of patients with SLE, but with new diagnostic tools it has been found that more people are affected. Central nervous system lupus is thought to actually be present in many lupus patients at some point in the course of the disease.
Treatments for CNS lupus involve anti-inflammatory and immunosuppressant drugs. High-dose oral or intravenous corticosteroids, such as methylprednisolone and prednisone, may be given. Immunosuppressants or cytotoxic drugs, such as cyclophosphamide, azathioprine, and methotrexate, may also be given. Anticoagulant drugs, such as warfarin, may be given to patients with antiphospholipid antibody syndrome or significant thrombosis. When the serum antibody load is high to the point that medical therapy is insufficient, artificial removal of these antibodies can be done through a process called plasmapheresis.
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