Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain.[1] Limbic encephalitis is caused by autoimmunity: an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not.[1] Although the disease is known as "limbic" encephalitis, it is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain.[2][3][4] The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968[3] and confirmed by later investigators.[5]
The majority of cases of limbic encephalitis are associated with a tumor (diagnosed or undiagnosed). In cases caused by tumor, recovery can only occur following complete removal of the tumor, which is not always possible. Limbic encephalitis is classified according to the auto-antibody that causes the disease. The most common types are:
Since 1999, following the publication of a case report of a 15-year-old teenager of Indian descent from South Africa who developed subacute memory loss subsequent to herpes simplex type 1 encephalitis,[6] similar cases of non-paraneoplastic LE have been described, as has its association with auto-antibodies and response to steroid.[7][8] Limbic encephalitis associated with voltage-gated potassium channel antibodies (VGKC-Abs)[9] may frequently be non-paraneoplastic.[10] A recent study of 15 cases of limbic encephalitis found raised VGKC-Abs associated with non-paraneoplastic disorders and remission following immunosuppressive treatment.[11]
^Brierley JB, Corsellis JA, Hierons R, et al. (1960). "Subacute encephalitis of later adult life. Mainly affecting the limbic areas". Brain. 83 (3): 357–368. doi:10.1093/brain/83.3.357.
^ abCorsellis JA, Goldberg GJ, Norton AR (1968). ""Limbic encephalitis" and its association with carcinoma". Brain. 91 (3): 481–496. doi:10.1093/brain/91.3.481. PMID5723018.