In this article we review various diagnostic techniques and therapeutic options for management of autoimmune encephalopathy. We also review medication adverse effects and monitoring strategies. Expert commentary: Early diagnosis and immunomodulatory treatment remains the cornerstone of management, to halt the underlying neuro-inflammatory process and prevent permanent neuronal injury. The availability of serological testing and various imaging modalities has further improved detection of these immune-mediated neurological disorders. Understanding the mechanisms and potential adverse effects of immunomodulatory therapies will help physicians to choose the most favorable therapeutic strategy for each patient.

A multitude of environmental factors can result in breakdown of immune tolerance in susceptible hosts. Infectious pathogens are among the most important environmental triggers in the pathogenesis of autoimmunity. Certain autoimmune disorders have a strong association with specific infections. Several neurological autoimmune disorders are thought to occur through post-infectious mechanisms. In this review, we discuss the proposed mechanisms underlying pathogen-induced autoimmunity, and highlight the clinical presentation and treatment of several post-infectious autoimmune neurological disorders. We also highlight post-infectious neurological disorders in the setting of recent outbreaks.

Background The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. In addition to neurological symptoms, a number of these disorders present with prominent gastrointestinal (GI) manifestations such as nausea, diarrhea, weight loss, and gastroparesis prompting an initial evaluation by gastroenterologists. Purpose This review provides a general overview of autoantibodies within the nervous system, focusing on three scenarios in which nervous system autoimmunity may initially present with gut symptoms. A general approach to evaluation and treatment, including antibody testing, will be reviewed.

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Introduction: Observational data suggest that B-cell-depleting therapies are effective for antibody-mediated autoimmune encephalitis. However, randomized controlled trials are needed. Here, we report challenges encountered in a randomized, placebo-controlled trial of ocrelizumab for autoimmune encephalitis that failed to meet recruitment goals. Methods: This was a single-center, 12-month, randomized, double-blind, placebo-controlled trial. Patients with autoimmune encephalitis were randomized in 1:1 fashion to placebo or ocrelizumab infusion after receiving first-line immunotherapy. The primary endpoint of the study was clinical worsening, defined as a perceived decline by the patient or clinician or a decrease in the Lawton and Brody Instrumental Activities of Daily Living Scale (IADL), along with either worsening on the Texas Functional Living Scale (TFLS) or hospitalization for symptoms of encephalitis.Results: Among 16 eligible patients, only three enrolled in the study, which closed due to poor recruitment. Two participants were randomized to the ocrelizumab arm and one to the placebo arm. The single patient in the placebo arm (NMDAR?) met the primary endpoint at 12 weeks and received open-label ocrelizumab with improvement. In the ocrelizumab arm, one participant (NMDAR?) demonstrated marked improvement, and the second (LGI1?) remained clinically stable. There were no serious adverse events associated with ocrelizumab. Conclusion: Clinical trial recruitment for autoimmune encephalitis is challenging, and our trial did not meet recruitment goals. Large, multicenter clinical trials are still needed, and careful attention must be given to study design, endpoints, and patient selection. Instrumented functional rating scales will be valuable outcome measures for future studies. Trial Registration: ClinicalTrials.gov identifier: NCT03835728.