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Limbic encephalitis


Title




Subtypes:
Limbic encephalitis with LGI1 antibodies

Summary Summary


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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]
Last updated: 4/26/2016

Symptoms Symptoms


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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]
Last updated: 5/11/2016
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Cause Cause


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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]
Last updated: 5/12/2016

Diagnosis Diagnosis


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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
Last updated: 8/5/2019

Treatment Treatment


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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]
Last updated: 5/12/2016

Prognosis Prognosis


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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.[1]

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.[2]
Last updated: 5/12/2016

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Encephalitis Society
    32 Castlegate
    Malton, North Yorkshire YO17 7DT
    United Kingdom
    Telephone: +44 (0)1653 699599
    E-mail: support@encephalitis.info
    Website: http://www.encephalitis.info/
    Social media: Twitter: @encephalitis Facebook: /encephalitissociety
  • International Autoimmune Encephalitis Society
    Telephone: 352-527-2470
    E-mail: IAES@autoimmune-encephalitis.org
    Website: https://autoimmune-encephalitis.org/

Social Networking Websites

  • The International Autoimmune Encephalitis Society Facebook group offers educational support through this forum.
Do you know of an organization? We want to hear from you.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The Encephalitis Society offers a factsheet on Limbic encephalitis
  • The International Autoimmune Encephalitis Society offers patients and families detailed information on diagnosis and treatment.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Limbic encephalitis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

  • Graus F, Titulaer MJ, Balu R, Benseler S et al., A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016 Apr;15(4):391-404.
  • Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR. LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. J Neurol Neurosurg Psychiatry. 2018 May;89(5):526-534.
  • Jammoul A, Li Y, Rae-Grant A. Autoantibody-­mediated encephalitis: Not just paraneoplastic, not just limbic, and not untreatable. Cleveland Clinic Journal of Medicine. 2016 January;83(1):43-53.

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Anderson NE, Barber PA. Limbic encephalitis - a review. Journal of Clinical Neuroscience. 2008; http://www.ncbi.nlm.nih.gov/pubmed/18411052.
  2. Asztely F, Kumlien E.. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. Dec 2012; 126(6):365-375. http://www.ncbi.nlm.nih.gov/pubmed/22713136.
  3. Dr Sarosh R Irani, Dr Camilla Buckley and Prof Angela Vincent. Limbic Encephalitis. The Encephalitis Society. August 2013; http://www.encephalitis.info/files/9413/3995/5695/FS013V1.pdf.
  4. Voltz R. Neuropsychological symptoms in paraneoplastic disorders. J Neurol. (2007); http://www.ncbi.nlm.nih.gov/pubmed/17503138.
  5. F Graus, J Delattre, J Antoine, J Dalmau, B Giometto, W Grisold, J Honnorat, P Smitt, C. Vedeler, J Verschuuren, A Vincent, R Voltz. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. August 2004; 75(8):1135-1140. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1739186/. Accessed 5/12/2016.
  6. Tuzun E, Dalmau J. Limbic Encephalitis and Variants: Classification, Diagnosis, and Treatment. The Neurologist. 2007;
Do you know of a review article? We want to hear from you.
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