Post-streptococcal opsoclonus-myoclonus syndrome associated with anti-neuroleukin antibodies

J Neurol Neurosurg Psychiatry. 2006 Apr;77(4):507-12. doi: 10.1136/jnnp.2005.078105.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antigens, Bacterial / blood
  • Antigens, Bacterial / cerebrospinal fluid
  • Antigens, Bacterial / immunology
  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid
  • Autoantibodies / immunology*
  • Autoantigens / blood
  • Autoantigens / cerebrospinal fluid
  • Autoantigens / immunology*
  • Bacterial Outer Membrane Proteins / immunology
  • Cell Membrane / immunology
  • Chromatography, Ion Exchange / methods
  • DNA Primers / genetics
  • DNA, Complementary / genetics
  • Electrophoresis, Polyacrylamide Gel / methods
  • Female
  • Glucose-6-Phosphate Isomerase / genetics
  • Glucose-6-Phosphate Isomerase / immunology*
  • Humans
  • Immunoblotting
  • Immunohistochemistry
  • Paraneoplastic Syndromes, Nervous System / diagnosis
  • Paraneoplastic Syndromes, Nervous System / immunology*
  • Paraneoplastic Syndromes, Nervous System / microbiology*
  • RNA, Messenger
  • Reverse Transcriptase Polymerase Chain Reaction
  • Streptococcal Infections / complications*
  • Streptococcal Infections / immunology*

Substances

  • Antigens, Bacterial
  • Autoantibodies
  • Autoantigens
  • Bacterial Outer Membrane Proteins
  • DNA Primers
  • DNA, Complementary
  • RNA, Messenger
  • Glucose-6-Phosphate Isomerase