Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
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
Other possible symptoms may include obsessiveness, hyperthermia (increase in body temperature), weight change, sleep disturbances, endocrine dysfunction, aphasia, and apraxia. 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.
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
• CSF pleocytosis (
3. Reasonable exclusion of alternative causes
Examples of conditions that must be ruled out before a diagnosis of limbic encephalitis can be made, include:
- 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
tumorof 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:
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.
- The International Autoimmune Encephalitis Society Facebook group offers educational support through this forum.
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.
- 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.
- 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.
- Anderson NE, Barber PA. Limbic encephalitis a review. Journal of Clinical Neuroscience. 2008; https://www.ncbi.nlm.nih.gov/pubmed/18411052.
- Asztely F, Kumlien E.. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. Dec 2012; 126(6):365-375. https://www.ncbi.nlm.nih.gov/pubmed/22713136.
- Dr Sarosh R Irani, Dr Camilla Buckley and Prof Angela Vincent. Limbic Encephalitis. The Encephalitis Society. August 2013; https://www.encephalitis.info/files/9413/3995/5695/FS013V1.pdf.
- Voltz R. Neuropsychological symptoms in paraneoplastic disorders. J Neurol. (2007); https://www.ncbi.nlm.nih.gov/pubmed/17503138.
- 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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1739186/. Accessed 5/12/2016.
- Tuzun E, Dalmau J. Limbic Encephalitis and Variants: Classification, Diagnosis, and Treatment. The Neurologist. 2007;