Clinical and Biological Predictors of Quality of Life in CASPR2-antibody Encephalitis
Bryan Ceronie1, Ikram Ayoub2, Christine Strippel1, Sophia Michael3, Chris Uy4, John Dawes1, David Bennett1, Sophie Binks1, Adam Handel1, Sarosh Irani2
1University of Oxford, 2Mayo Clinic, 3Birmingham, Queen Elizabeth Hospital, 4Department of Neurology & Neuroimmunology, University of British Columbia
Objective:

To assess the clinical and biological features that predict quality of life outcomes after immunotherapy in CASPR2-antibody disease.

Background:

CASPR2-antibody disease is an autoantibody-mediated neurological disease which may present as limbic encephalitis, Morvan’s Syndrome or isolated peripheral nerve hyperexcitability (PNH). To date, there have been conflicting reports about the factors associated with worse clinical outcomes. Neuropathic pain (NeuP) and fatigue are important drivers of disability and worsened quality of life (QoL), but their degree of response to immunotherapy is contested.

Design/Methods:

75 patients with CASPR2-antibody seropositivity and a relevant clinical syndrome were included. Outcome measures including symptom persistence, EuroQoL-5D-5L QoL scores, modified Rankin scale (mRS) and pain measures were collected retrospectively over a 6-year study period. Patient sera across multiple time points were interrogated by live cell-based assay and flow cytometry-based immunoglobulin G subclass assays. Clinical and biological predictors were assessed with multiple logistic regression and univariate analysis.

Results:

Despite most symptoms responding to immunotherapy in the first 6-12 months, NeuP and fatigue emerged as the most prominent and persistent symptoms, associated with a worse clinical trajectory of recovery and predicting worse disability, QoL and risk of relapse.

 

While other QoL domains improved, pain scores did not change over time. IgG1 levels were higher in the LE cohort (p<0.001) and NeuP was associated with lower IgG1 levels in the early phase (<12 months) of the disease (p=0.01). The presence of the human leukocyte antigen (HLA) DRB1:11*01 allele was associated with less NeuP (p=0.001) and better QoL (p=0.01) at 2 years.

Conclusions:

NeuP and fatigue are prominent residual features of CASPR2-antibody disease which do not respond to immunotherapy. IgG subclass analysis suggests IgG1 may have a more limbic predilection, versus a more peripheral, IgG4-mediated effect in causing NeuP. HLA genotyping may be a useful biomarker to predict clinical outcomes.

10.1212/WNL.0000000000215971
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