Leucine-rich Glioma-inactivated 1 Encephalitis Followed by Isaacs Syndrome: Alternating Presence of Pathogenic Autoantibodies to Leucine-rich Glioma-inactivated 1 and Contactin-associated Protein-like 2.

Abstract

The coexistence of leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) autoantibodies in the same individual is surprisingly often observed. We herein report the first case of LGI1 encephalitis followed by Isaacs syndrome in which LGI1 and CASPR2 antibodies in the serum and cerebrospinal fluid (CSF) were measured during the entire disease course. After the resolution of limbic encephalitis, LGI1 antibodies disappeared from the CSF simultaneously with the appearance of CASPR2 antibodies in the serum. The alternating presence of these pathogenic autoantibodies along with the clinical and phenotypic alternations suggested that LGI1 encephalitis was associated with CASPR2 autoantibody production in the peripheral tissue, leading to CASPR2-associated Isaacs syndrome.

• Authors: Aki Murayama, Keizo Sugaya, Makoto Hara, Tomoya Kawazoe, Shinsuke Tobisawa, Hideto Nakajima, Kazushi Takahashi
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 62 Issue 11 Pg. 1659-1663 (Jun 01 2023) ISSN: 1349-7235 [Electronic] Japan
• PMID: 36223927 (Publication Type: Case Reports, Journal Article)

Chemical References

• Autoantibodies
• Leucine
• Intracellular Signaling Peptides and Proteins
• Contactins

Topics

• Humans
• Autoantibodies
• Leucine
• Isaacs Syndrome (complications)
• Intracellular Signaling Peptides and Proteins
• Encephalitis (diagnosis, complications)
• Limbic Encephalitis (complications)
• Contactins