Publications by authors named "C Geis"
Introduction: Very rarely, adult NMDAR antibody-associated encephalitis (NMDAR-E) leads to persistent cerebellar atrophy and ataxia. Transient cerebellar ataxia is common in pediatric NMDAR-E. Immune-mediated cerebellar ataxia may be associated with myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP-4), kelch-like family member 11 (KLHL11), and glutamate kainate receptor subunit 2 (GluK2) antibodies, all of which may co-occur in NMDAR-E.
View Article and Find Full Text PDFLambert-Eaton myasthenic syndrome (LEMS) is an autoantibody-mediated disease of the neuromuscular junction characterized by muscular weakness. Autoantibodies to presynaptic P/Q-type voltage-gated calcium channels (VGCCs) induce defective neuromuscular function. In severe cases, current immunosuppressive and immunomodulatory treatment strategies are often insufficient.
View Article and Find Full Text PDFArticle Synopsis
- Eculizumab (ECU) has shown effectiveness in preventing attacks in patients with aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorders (NMOSDs) during a retrospective analysis in clinical settings between 2014 and 2022.
- A total of 52 patients were studied, with 88% being attack-free during treatment, and the annualized attack rate significantly decreased from 1.0 to 0.
- While common side effects included serious infections, five patients died from various complications, indicating a need for careful monitoring during long-term ECU therapy.
Structural and functional mechanisms of anti-NMDAR autoimmune encephalitis.
Nat Struct Mol Biol
December 2024
Autoantibodies against neuronal membrane proteins can manifest in autoimmune encephalitis, inducing seizures, cognitive dysfunction and psychosis. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most dominant autoimmune encephalitis; however, insights into how autoantibodies recognize and alter receptor functions remain limited. Here we determined structures of human and rat NMDARs bound to three distinct patient-derived antibodies using single-particle electron cryo-microscopy.
View Article and Find Full Text PDFObjectives: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder that can be associated with antibodies against surface antigens (glycine receptor (GlyR), dipeptidyl-peptidase-like-protein-6) and intracellular antigens (glutamate decarboxylase (GAD65), amphiphysin).
Methods: We report clinico-pathologic findings of a PERM patient with coexisting GlyR and GAD65 antibodies.
Results: A 75-year-old man presented with myoclonus and pain of the legs, subsequently developed severe motor symptoms, hyperekplexia, a pronounced startle reflex, hallucinations, dysautonomia, and died 10 months after onset despite extensive immunotherapy, symptomatic treatment, and continuous intensive care support.