Mechanisms underlying autoimmune synaptic encephalitis leading to disorders of memory, behavior and cognition: insights from molecular, cellular and synaptic studies

Eur J Neurosci. 2010 Jul;32(2):298-309. doi: 10.1111/j.1460-9568.2010.07349.x. Epub 2010 Jul 14.

Abstract

Recently, several novel, potentially lethal and treatment-responsive syndromes that affect hippocampal and cortical function have been shown to be associated with auto-antibodies against synaptic antigens, notably glutamate or GABA-B receptors. Patients with these auto-antibodies, sometimes associated with teratomas and other neoplasms, present with psychiatric symptoms, seizures, memory deficits and decreased levels of consciousness. These symptoms often improve dramatically after immunotherapy or tumor resection. Here we review studies of the cellular and synaptic effects of these antibodies in hippocampal neurons in vitro and preliminary work in rodent models. Our work suggests that patient antibodies lead to rapid and reversible removal of neurotransmitter receptors from synaptic sites, leading to changes in synaptic and circuit function that in turn are likely to lead to behavioral deficits. We also discuss several of the many questions raised by these and related disorders. Determining the mechanisms underlying these novel anti-neurotransmitter receptor encephalopathies will provide insights into the cellular and synaptic bases of the memory and cognitive deficits that are hallmarks of these disorders, and potentially suggest avenues for therapeutic intervention.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Autoantibodies / immunology
  • Autoantigens / immunology
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / physiopathology*
  • Cognition Disorders / immunology
  • Cognition Disorders / physiopathology*
  • Encephalitis / immunology
  • Encephalitis / physiopathology*
  • Humans
  • Memory Disorders / immunology
  • Memory Disorders / physiopathology*
  • Synapses / immunology*

Substances

  • Autoantibodies
  • Autoantigens