Limbic encephalitis: what is it?

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Limbic encephalitis is inflammation of the limbic system caused by infection or autoimmune response. It can lead to memory loss, confusion, and seizures. Treatment depends on the cause, with antivirals or immunosuppressive drugs often used. PLE is associated with certain cancers.

Limbic encephalitis is a disease characterized primarily by inflammation of the limbic system, which is a group of structures located at the base of the brain. The problem can occur for one of two main reasons. The first is infection, because some viruses tend to target this part of the brain. The other is the result of an autoimmune response, in which the body’s immune system attacks it. This category can be further divided into two types: paraneoplastic limbic encephalitis, or PLE, and nonparaneoplastic limbic encephalitis, or NPLE.

An infection in the brain that attacks the limbic system can lead to limbic encephalitis. Although there are several viruses that could be the cause, the most common is the herpes simplex virus. The condition is sometimes known as herpes simplex encephalitis for this reason.

Paraneoplastic limbic encephalitis, or PLE, typically occurs in patients with certain types of cancer. Antibodies produced by the body to fight cancer can mistakenly attack structures in the limbic system, leading to inflammation. The most common type of cancer that leads to this situation is small cell carcinoma of the lungs, but it can also occur with cancers of the stomach, breast, testicles, or thymus gland.

In cases of nonparaneoplastic limbic encephalitis, the limbic structures of the brain are also attacked by antibodies, but they are not generated as an immune response to cancer in the body. Typically, these antibodies target and destroy proteins called potassium channels, which reduce control over how electrical signals travel through the brain. Another name for this problem is voltage-gated potassium channel antibody-associated encephalitis.

Regardless of the underlying cause, patients with limbic encephalitis typically experience similar symptoms. Memory loss is common, as the limbic system is central to forming memories. Some patients may become confused or disoriented, or may even show signs of delirium. In some cases, the disorder can trigger seizures. Brain scans done with an MRI often show changes in brain signals associated with limbic structures.

Treatment of limbic encephalitis depends on the cause of the disease. Viral infections can be treated with antiviral medications to help clear the infection. In cases where an autoimmune response is to blame, immunosuppressive drugs can often be helpful. For those with PLE, treating the cancer causing antibodies also often play a role.




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