Neural antigen-specific autoimmune disorders

Raffaele Iorio, Vanda A Lennon

Research output: Contribution to journalArticle

51 Citations (Scopus)

Abstract

Neural-specific autoantibodies have been documented and their diagnostic utility validated in diseases affecting the neuraxis from cerebral cortex to the somatic, autonomic, and enteric nervous system and skeletal muscle. These neurological disorders occur both idiopathically and in a paraneoplastic context. Molecular identification of the antigens has expedited development of confirmatory and high-throughput tests for serum and cerebrospinal fluid, which permit early diagnosis and reveal the underlying molecular pathogenic mechanisms. The autoantibodies are classifiable on the basis of antigen location: intracellular (nuclear or cytoplasmic) or plasma membrane. Immunohistopathological studies of patients' biopsied and autopsied tissues suggest that effector T cells mediate the autoimmune neurological disorders for which defining autoantibodies recognize intracellular antigens. Antigens within intact cells are inaccessible to circulating antibody, and the associated neurological deficits rarely improve with antibody-depleting therapies. Tumoricidal therapies may arrest neurological progression, but symptom reversal is rare. In contrast, autoantibodies specific for plasma membrane antigens have pathogenic potential, and the associated neurological deficits are often amenable to antibody-depleting immunotherapy, such as plasma exchange and anti-B-cell monoclonal antibody therapy. These reversible neurological disorders are frequently misdiagnosed as neurodegenerative. The focus of this review is the immunobiology, pathophysiology, and clinical spectrum of autoimmune neurological disorders accompanied by neural-specific IgGs.

Original languageEnglish (US)
Pages (from-to)104-121
Number of pages18
JournalImmunological Reviews
Volume248
Issue number1
DOIs
StatePublished - Jul 2012

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Nervous System Diseases
Autoantibodies
Antigens
Cell Membrane
Antibodies
Enteric Nervous System
Plasma Exchange
Autonomic Nervous System
Diagnostic Errors
Cerebral Cortex
Immunotherapy
Cerebrospinal Fluid
Early Diagnosis
Skeletal Muscle
B-Lymphocytes
Therapeutics
Monoclonal Antibodies
T-Lymphocytes
Serum

Keywords

  • Autoantibodies
  • Paraneoplastic neurological diseases

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Neural antigen-specific autoimmune disorders. / Iorio, Raffaele; Lennon, Vanda A.

In: Immunological Reviews, Vol. 248, No. 1, 07.2012, p. 104-121.

Research output: Contribution to journalArticle

Iorio, Raffaele ; Lennon, Vanda A. / Neural antigen-specific autoimmune disorders. In: Immunological Reviews. 2012 ; Vol. 248, No. 1. pp. 104-121.
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