Autoantibody Encephalitis: Presentation, Diagnosis, and Management

J Clin Neurol. 2022 Jul;18(4):373-390. doi: 10.3988/jcn.2022.18.4.373.

Abstract

Autoantibody encephalitis causes distinct clinical syndromes involving alterations in mentation, abnormal movements, seizures, psychiatric symptoms, sleep disruption, spasms, and neuromyotonia. The diagnoses can be confirmed by specific antibody tests, although some antibodies may be better detected in spinal fluid and others in serum. Each disorder conveys a risk of certain tumors which may inform diagnosis and be important for treatment. Autoantibodies to receptors and other neuronal membrane proteins are generally thought to be pathogenic and result in loss of function of the targets, so understanding the pharmacology of the receptors may inform our understanding of the syndromes. Patients may be profoundly ill but the syndromes usually respond to immune therapy, although there are differences in the types of immune therapy that are thought to be most effective for the various disorders.

Keywords: anti-NMDAR encephalitis; antibody; cerebellar ataxia; encephalitis; paraneoplastic.

Publication types

  • Review