VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy.

Abstract

Leucine-rich glioma inactivated 1 (LGI1) was recently identified as a target protein in autoimmune synaptic encephalitis, a rare condition associated with autoantibodies against structures in the neuronal synapse. Studies dealing with LGI1 are small in number and the various outcomes of different therapeutic regimens are not well studied. Here, we analyzed clinical characteristics of 14 patients with LGI1 antibodies, and outcomes according to therapeutic strategies. Most patients exhibited abnormal brain positron emission tomography and that patients treated with steroids alone were more likely to relapse and had less favorable outcomes than those treated with steroids and intravenous immunoglobulins.

• Authors: Yong-Won Shin, Soon-Tae Lee, Jung-Won Shin, Jangsup Moon, Jung-Ah Lim, Jung-Ick Byun, Tae-Joon Kim, Keon-Joo Lee, Young-Su Kim, Kyung-Il Park, Keun-Hwa Jung, Sang Kun Lee, Kon Chu
• Journal: Journal of neuroimmunology (J Neuroimmunol) Vol. 265 Issue 1-2 Pg. 75-81 (Dec 15 2013) ISSN: 1872-8421 [Electronic] Netherlands
• PMID: 24176648 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2013.

Chemical References

• Antibodies
• Autoantibodies
• Immunoglobulins, Intravenous
• Intracellular Signaling Peptides and Proteins
• LGI1 protein, human
• Proteins
• Fluorodeoxyglucose F18

Topics

• Adult
• Aged
• Antibodies (blood)
• Autoantibodies (blood)
• Brain (diagnostic imaging, metabolism, pathology)
• Brain Diseases (blood, diagnostic imaging, immunology, therapy)
• Electroencephalography
• Encephalitis
• Female
• Fluorodeoxyglucose F18
• Hashimoto Disease (blood, diagnostic imaging, immunology, therapy)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunotherapy (methods)
• Intracellular Signaling Peptides and Proteins
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Positron-Emission Tomography
• Proteins (immunology)
• Treatment Outcome