Anti-contactin-associated protein-like 2 antibody-associated cerebellar ataxia: A case report and literature review

J Neuroimmunol. 2021 Apr 15;353:577515. doi: 10.1016/j.jneuroim.2021.577515. Epub 2021 Feb 3.

Abstract

The spectrum of anti-contactin-associated protein-like 2 (CASPR2) antibody-associated disease is expanding and the involvement of cerebellum was reported in the past few years. We report a 45-year-old male with chronically progressive cerebellar ataxia. CASPR2 antibodies were detected in his serum and cerebellar atrophy was observed on MRI. His symptoms improved prominently with steroids and intravenous immunoglobulins. 23 cases with CASPR2 antibodies and cerebellar ataxia were identified from previous publications. Most of patients showed acute or subacute onset with other typical presentations of anti-CASPR2 antibody-associated disease, such as limbic encephalitis. Immunotherapy was effective in the majority of patients.

Keywords: Autoimmune encephalitis; Cerebellar ataxia; Contactin associated protein-like 2.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Atrophy / immunology
  • Atrophy / pathology
  • Autoantibodies / immunology*
  • Autoantigens / immunology
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Cerebellar Ataxia / drug therapy
  • Cerebellar Ataxia / immunology*
  • Cerebellar Ataxia / pathology
  • Humans
  • Immunologic Factors / therapeutic use
  • Immunotherapy / methods
  • Male
  • Membrane Proteins / immunology*
  • Middle Aged
  • Nerve Tissue Proteins / immunology*

Substances

  • Autoantibodies
  • Autoantigens
  • CNTNAP2 protein, human
  • Immunologic Factors
  • Membrane Proteins
  • Nerve Tissue Proteins