Limbic Encephalitis – Causes, Symptoms, Diagnosis, Treatment

Dr. Harun Ar Rashid, MD - Arthritis, Bones, Joints Pain, Trauma, and Internal Medicine Specialist Dr. Harun Ar Rashid, MD - Arthritis, Bones, Joints Pain, Trauma, and Internal Medicine Specialist
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Rx Autoimmune, Genetic and Rare Diseases (A - Z)
Limbic encephalitis represents a group of autoimmune conditions characterized by inflammation of the limbic system and other parts of the brain. The cardinal sign of limbic encephalitis is a severe impairment of short-term memory; however, symptoms may also include confusion, psychiatric symptoms, and seizures. The symptoms typically develop over a few weeks or months, but they may evolve over a few days. Limbic encephalitis is often associated with an underlying neoplasm (paraneoplastic limbic encephalitis); however some cases never have a neoplasm identified (non-paraneoplastic limbic encephalitis). Delayed diagnosis is common, but improvements are being made to assist in early detection. Various tests including imaging studies (MRI, PET) laboratory tests (CSF analysis), and tests that measure the electrical activity of the brain (EEG) may be utilized to confirm a diagnosis. Treatment includes removal of the neoplasm (if identified) and immunotherapy.[1][2][3]

Although the symptoms of the condition may vary from person to person, the presenting sign of limbic encephalitis is severe impairment of short-term memory, with most patients having difficulties in recall. Epileptic seizures are common and may occur prior to symptoms of memory loss.[2] A variety of symptoms may be associated with limbic encephalitis such as anterograde amnesia (the inability to store new memories after the onset of the condition), anxiety, depression, irritability, personality change, acute confusional state, hallucinations and seizures.

Other possible symptoms may include obsessiveness, hyperthermia (increase in body temperature), weight change, sleep disturbances, endocrine dysfunction, aphasia, and apraxia.[4][2] The symptoms associated with limbic encephalitis can develop over a few days, weeks, or months. It is important to note the neurological symptoms generally precede diagnosis of the malignancy in 60%-75% of patients that have paraneoplastic limbic encephalitis.[1]

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.[3] 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.[4][2] 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.[2][3]

Diagnosis of limbic encephalitis can be made when all three of the following criteria have been met:[14588]

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:[5]

  • Infection (herpes simplex virus, cytomegalovirus, adenovirus, HIV)
  • Acute disseminating encephalomyelitis (ADEM)
  • Degenerative diseases (Creutzfeldt-Jakob disease, rapidly progressing Alzheimer disease)
  • Nutritional deficiencies (thiamine deficiency)
  • Recreational drugs/alcohol
  • Metabolic disturbances
  • Connective tissue disorders (systemic lupus erythematosus)
  • Primary or secondary tumor of the central nervous system

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:
https://autoimmune-encephalitis.org/diagnosis

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.[6][2] If immunotherapy fails, medications such as rituximab or cyclophosphamide may be considered.[2]

 

References

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