J Clin Neurol.  2024 Jan;20(1):3-22. 10.3988/jcn.2023.0242.

Outcome and Sequelae of Autoimmune Encephalitis

Affiliations
  • 1Department of Neurology & Neurological Sciences, Center for Academic Medicine, Stanford University, Stanford, CA, USA
  • 2Grenoble Alpes University, Grenoble, France
  • 3Department of Neurology, Weill Institute for Neurosciences, University of California, San Francisco, CA, USA
  • 4Department of Medicine, Division of Infectious Diseases, University of California, San Francisco, CA, USA
  • 5National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
  • 6Infectious Diseases and Intensive Care Unit, Pontchaillou University Hospital, Rennes, France
  • 7Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA, USA
  • 8Department of Infectious Diseases, Santé publique France, Saint-Maurice, France

Abstract

Autoimmune etiologies are a common cause for encephalitis. The clinical syndromes consistent with autoimmune encephalitis are both distinct and increasingly recognized, but less is known about persisting sequelae or outcomes. We searched PubMed for reports on outcomes after autoimmune encephalitis. Studies assessing validated, quantitative outcomes were included. We performed a narrative review of the published literature of outcomes after autoimmune encephalitis. We found 146 studies that produced outcomes data. The mortality rates were 6%–19% and the relapse risks were 10%–62%. Most patients achieved a good outcome based on a score on the modified Rankin Scale (mRS) of ≤2. Forty-nine studies evaluated outcomes beyond mRS; these studies investigated cognitive outcome, psychiatric sequelae, neurological deficits, global function, and quality-of-life/patient-reported outcomes using various tools at varying time points after the index hospital discharge. These more-detailed assessments revealed that most patients had persistent impairments, with frequent deficits in cognitive function, especially memory and attention. Depression and anxiety were also common. Many of these sequelae continued to improve over months or even years after the acute illness. While we found that lasting impairments were common among survivors of autoimmune encephalitis, additional research is needed to better understand the nature and impact of these sequelae. Standardized evaluation protocols are needed to improve the ability to compare outcomes across studies, guide rehabilitation strategies, and inform outcomes of interest in treatment trials as the field advances.

Keyword

autoimmune encephalitis; outcomes; cognitive impairment; patient-reported outcome
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