Topic: anti-IgLON5 disease

Anti-IgLON5 disease is a rare autoimmune disorder with IgLON5 autoantibodies predominantly of the IgG4 subclass. The clinical relevance of the pathogenic effects of anti-IgLON5 IgG1 and IgG4 is unclear. IgLON5-specific IgG1-4 levels were measured in 46 sera and 20 cerebrospinal fluid (CSF) samples from 13 HLA-subtyped anti-IgLON5 disease patients (6 female, 7 male) using flow cytometry. Intervals between two consecutive serum or CSF samplings were analyzed for treatment, changes of anti-IgLON5 IgG1/IgG4 levels and disease severity. Intrathecal anti-IgLON5 IgG4 synthesis (IS) was assessed. Th

Anti-IgLON5 is a rare neurological disorder characterized by brainstem dysfunction, abnormal movements, sleep alterations, autoantibodies against IgLON5, and neurodegeneration, sometimes with neuronal deposits of tau. IgLON5 is a neural cell adhesion molecule of unknown function. This study was aimed to determine the effects of loss of function of IgLON5 in a Knockout (IgLON5-KO) mouse model assessing brain morphology, social behavior, and symptoms.IgLON5-KO mouse was produced by CRISPR-Cas9 technology. Two-month-old IgLON5-KO and wild-type mice were subjected to a comprehensive battery of beh