Limbic encephalitis

What causes limbic encephalitis?

Limbic encephalitis is thought to be caused by a reaction of the immune system to various stimuli such as cancers, tumors, infections, and generalized autoimmune disorders. In many patients, limbic encephalitis is associated with a tumor or cancer. This is known as paraneoplastic limbic encephalitis (PLE). PLE is most commonly associated with small cell lung cancer (SCLC), which is present in 40% of cases. It may additionally be associated with breast cancer, testicular cancer, and nearly any other tumor. Limbic encephalitis can also occur in the absence of cancer, known as non-paraneoplastic limbic encephalitis (NPLE), such as in the case of a viral infection (for example herpes simplex virus) or systemic autoimmune disorders. In some instances, the cause of limbic encephalitis may never be determined.

Last updated on 05-01-20

How is limbic encephalitis diagnosed?

Diagnosis of limbic encephalitis can be made when all three of the following criteria have been met:

1. Subacute onset (rapid progression of less than 3 months) of working memory deficits (short-term memory loss), altered mental status, or psychiatric symptoms

2. At least one of the following:

• New focal CNS findings
• Seizures not explained by a previously known seizure disorder
• CSF pleocytosis (white blood cell count of more than five cells per mm3)
• MRI features suggestive of encephalitis

3. Reasonable exclusion of alternative causes

Examples of conditions that must be ruled out before a diagnosis of limbic encephalitis can be made, include:

A team of leading autoimmune encephalitis researchers have established diagnostic criteria for limbic encephalitis. View the full position paper, entitled A Clinical Approach to Diagnosis of Autoimmune Encephalitis.

For more on diagnosis, visit the following link to the International Autoimmune Encephalitis Society Web site:

Last updated on 05-01-20

What is the long-term outlook for limbic encephalitis?

The prognosis for limbic encephalitis varies from person to person and is based on the underlying cause of the condition. For example, individuals that have small cell lung carcinoma (SCLC) and test positive for an antibody called anti-Hu, tend to respond poorly to treatment, whereas individuals that have an ovarian teratoma and test positive for the NMDAR antibody respond well to treatment.

Given evidence that paraneoplastic limbic encephalitis may occur prior to identification of a tumor, if a tumor is not initially identified, regular tumor screening may be recommended to occur after treatment is completed.

Last updated on 05-01-20

How might limbic encephalitis be treated?

Treatment for limbic encephalitis varies depending on the underlying cause. In cases of confirmed paraneoplastic limbic encephalitis (PLE), removal or treatment of the tumor is often the first step in treatment. If the cause of limbic encephalitis is a viral infection, an antiviral drug may be prescribed. Immunotherapy is often utilized as a first line or second line treatment. If immunotherapy fails, medications such as rituximab or cyclophosphamide may be considered.

Last updated on 05-01-20

Name: Encephalitis Society 32 Castlegate Malton, North Yorkshire YO17 7DT
United Kingdom
Phone: +44 (0)1653 699599 Email: Url: Social media: Twitter: @encephalitis Facebook: /encephalitissociety
Name: International Autoimmune Encephalitis Society United States Phone: 352-527-2470 Email: Url:

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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