自己免疫性脳炎・脳症とアフェレシス

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  • Autoimmune Encephalitis/Encephalopathy and Apheresis

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Autoimmune encephalitis/encephalopathy producing disease-specific autoantibodies has been increasingly recognized with newly developed antibody-detection systems. Among its varieties, those with antibodies against neuronal cell surface functional proteins such as anti-aquaporin 4 antibody-related neuromyelitis optica and anti-NMDA receptor antibody-related limbic encephalitis are recently designated groups. These with antibodies against cell surface proteins are usually well responsive to immune therapy such as plasmapheresis or antibody-deprivation therapies. On the other hand, those with antibodies against intracellular proteins detected in most of the paraneoplastic neurological syndromes tend to be resistant to immune therapies whose pathogenic effectors are thought to be cytotoxic T cells. However, the detection of these specific antibodies is extremely important for the proper diagnosis of autoimmune encephalitis/encephalopathy, a group of wide range of variable neurological symptoms not easy to be diagnosed only with clinical features. Some of the antibodies had proven to have direct effects on neuronal dysfunctions and cause their symptoms, and therefore would be proper candidates for treatment with apheresis.

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