Article Text

PDF
Post-streptococcal opsoclonus-myoclonus syndrome associated with anti-neuroleukin antibodies
  1. P M Candler1,
  2. R C Dale1,
  3. S Griffin3,
  4. A J Church1,
  5. R Wait2,
  6. M D Chapman1,
  7. G Keir1,
  8. G Giovannoni1,
  9. J H Rees1
  1. 1Department of Neuroinflammation, Institute of Neurology, Queen Square, London, WC1N 3BG, UK
  2. 2The Kennedy Institute of Rheumatology, Imperial College, London, UK
  3. 3Department of Neurochemistry, Institute of Neurology, Queen Square, London, WC1N 3BG, UK
  1. Correspondence to:
 Paul M Candler
 Department of Neuroinflammation, Institute of Neurology, Queen Square, London, WC1N 3BG, UK;p.candler{at}ion.ucl.ac.uk

Abstract

Background: Adult opsoclonus-myoclonus (OM), a disorder of eye movements accompanied by myoclonus affecting the trunk, limbs, or head, is commonly associated with an underlying malignancy or precipitated by viral infection.

Methods: We present the first two reports of post-streptococcal OM associated with antibodies against a 56 kDa protein. Two young girls presented with opsoclonus and myoclonus following a febrile illness and pharyngitis. Protein purification techniques were employed. Amino acid sequences of human neuroleukin (NLK) and streptococcal proteins were compared using the protein-protein BLAST application.

Results: The antigen was identified as NLK (glucose-6-phosphate isomerase, GPI). GPI is present on the cell surface of streptococcus making the protein a candidate target for molecular mimicry.

Conclusions: We have identified NLK as an antigenic target in two patients with post-streptococcal OM. The pathogenicity of the antibodies is uncertain. The potential role of anti-neuroleukin antibodies in the pathogenesis of OM is discussed. We propose that OM may represent a further syndrome in the growing spectrum of post-streptococcal neurological disorders. The role of streptococcus in OM and the frequency with which anti-NLK responses occur in both post-infectious and paraneoplastic OM should be investigated further.

  • ASOT, anti-streptolysin-O titre
  • GPI, glucose-6-phosphate isomerase
  • HVA, homovanillic acid
  • IEX, ion exchange chromatography
  • NLK, neuroleukin
  • OM, opsoclonus-myoclonus
  • PAGE, polyacrylamide gel electrophoresis
  • rNLK, recombinant neuroleukin
  • TBS, Tris-buffered saline
  • VMA, vanillylmandelic acid
  • antibodies
  • opsoclonus-myoclonus
  • post-infectious
  • streptococcus

Statistics from Altmetric.com

Footnotes

  • Competing interests: none declared

  • Patient details are published with consent

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.