A case of severe chronic progressive axonal polyradiculoneuropathy temporally associated with anti-CV2/CRMP5 antibodies

J Clin Neuromuscul Dis. 2013 Sep;15(1):13-8. doi: 10.1097/CND.0b013e3182a04538.

Abstract

Objectives: The involvement of the peripheral nervous system by anti-CV2/CRMP5 paraneoplastic antibodies is typically encountered as a mixed sensorimotor polyneuropathy. We report a fatal case of severe chronic progressive axonal polyradiculoneuropathy in association with this antibody.

Methods: Review of the patient's chart, nerve conduction/electromyographic studies, and nerve biopsy.

Results: A 51-year-old man presented with a progressive quadriparesis over a 4-month period. Extensive evaluation for potential etiologies was significant only for positive anti-CV2/CRMP5 antibodies without detection of an underlying neoplasm. Despite multiple immunomodulatory therapies, the patient progressed and demonstrated electrodiagnostic evidence for a chronic axonal polyradiculoneuropathy with ongoing denervation. The patient eventually died of respiratory failure.

Conclusions: This case adds to the clinical spectrum of the peripheral nervous system involvement in patients with paraneoplastic anti-CV2/CRMP5 antibodies.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / immunology*
  • Autoantigens / immunology
  • Fatal Outcome
  • Humans
  • Hydrolases
  • Male
  • Microtubule-Associated Proteins
  • Middle Aged
  • Nerve Tissue Proteins / immunology*
  • Polyradiculoneuropathy / immunology*
  • Polyradiculoneuropathy / pathology*

Substances

  • Autoantibodies
  • Autoantigens
  • Microtubule-Associated Proteins
  • Nerve Tissue Proteins
  • DPYSL5 protein, human
  • Hydrolases