Paraneoplastic anti-CV2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy

Ann Neurol. 2001 Feb;49(2):214-21. doi: 10.1002/1531-8249(20010201)49:2<214::aid-ana41>;2-w.


Subacute sensory neuronopathy with anti-Hu antibodies is the best-characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti-CV2 antibodies, another group of paraneoplastic antibodies, react with a 66-kd brain protein belonging to the family of Ulip/CRMP proteins. The manifestations associated with anti-CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti-Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti-CV2 antibodies (3 of whom also had anti-Hu antibodies) and 12 patients with only anti-Hu antibodies. Data for patients with anti-Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti-CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti-CV2 and anti-Hu antibodies were present. Unlike anti-Hu antibodies, anti-CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66-kd protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Antibodies, Anti-Idiotypic / immunology*
  • Axons / immunology*
  • Demyelinating Diseases / immunology*
  • Demyelinating Diseases / pathology
  • Demyelinating Diseases / physiopathology
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Paraneoplastic Polyneuropathy / immunology*
  • Paraneoplastic Polyneuropathy / pathology
  • Paraneoplastic Polyneuropathy / physiopathology
  • Peripheral Nerves / immunology*


  • Antibodies, Anti-Idiotypic