National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Limbic encephalitis with LGI1 antibodies


Other Names:
Limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies
Categories:
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Limbic encephalitis with LGI1 antibodies is a relatively new disease characterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation. Seizures are common and frequently precede other symptoms. About half of people with this diagnosis exhibit a specific type of seizure called faciobrachial dystonic seizures, which resemble brief jerks of the face arm.[1][2][3][4][5][6] Hyponatremia (low levels of sodium in the blood) may also be present.[1][3][6] The disease occurs when antibodies mistakenly attack LGI1 (leucine-rich glioma inactivated 1), a protein found in the brain that binds together two epilepsy-related proteins called ADAM22 and ADAM23.[1][5][6] Some people with limbic encephalitis with LGI1 antibodies (5 to 10%) also have a cancer diagnosis (most commonly thymoma and lung cancer), but most do not.[1][6] 

The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies.[1][3][5] If treatment is started when seizures are first noticed, limbic encephalitis may be prevented.[1][2] Treatment is intravenous or oral corticosteroids, intravenous immunoglobulin (IVIG), plasmapheresis (a process where antibodies are removed from the blood), or a combination of these.[1][3][5][6] Long-term outcome is mostly favorable, although some people are left with some degree of residual impairment. Relapse occurs in up to one third of patients.[1][5][7][8]
Last updated: 12/1/2017

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In-Depth Information

  • 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 with LGI1 antibodies. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

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  1. van Sonderen A, Schreurs MW, Wirtz PW, Sillevis Mitt PA, Titulaer MJ. From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time. Autoimmun Rev. 2016 Oct; 15(10):970-4. http://www.sciencedirect.com/science/article/pii/S156899721630163X?via%3Dihub.
  2. Belmer NJ, Selwa LM. Seizure semiology of anti-LGI1 antibody encephalitis. Epileptic Disord. 2017 Nov 24; https://www.ncbi.nlm.nih.gov/pubmed/29171405.
  3. Yu J, Yu X, Fang S, Zhang Y, Lin W. The Treatment and Follow-Up of Anti-LGI1 Limbic Encephalitis. Eur Neurol. 2016; 75(1-2):5-11. https://www.ncbi.nlm.nih.gov/pubmed/26694143.
  4. Lee JJ, Lee ST, Jung KH, Chu K, Lee SK. Anti-LGI1 Limbic Encephalitis Presented with Atypical Manifestations. Exp Neurobiol. 2013 Dec; 22(4):337-340. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3897696/.
  5. Irani S. LGI1/CASPR2-Antibody Encephalitis. Encephalitis Society. September 2017; https://www.encephalitis.info/lgi1caspr2-antibody-encephalitis.
  6. Dalmau J. Paraneoplastic and autoimmune encephalitis. UpToDate. July 26, 2017; https://www.uptodate.com/contents/paraneoplastic-and-autoimmune-encephalitis.
  7. van Sonderen A, THijs RD, Voenders EC, Jiskoot LC, Sanchez E, se Bruijn MA, van Coevorden-Hameete MH, Wirtz PW, Schreurs MW, Sillevis Smitt PA, Titulaer MJ. Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up. Neurology. 2016 Oct 4; 87(14):1449-1456. https://www.ncbi.nlm.nih.gov/pubmed/27590293.
  8. Ariño H, Armangué T, Petit-Pedrol M, Sabater L, Martinez-Hernandez E, Hara M, Lancaster E, Saiz A, Dalmau J, Graus F. Anti-LGI1–associated cognitive impairment: Presentation and long-term outcome. Neurology. 2016 Aug 23; 87(8):759-65. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4999321/.