Post-streptococcal opsoclonus-myoclonus syndrome associated with anti-neuroleukin antibodies.
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.
Department of Neuroinflammation, Institute of Neurology, Queen Square, London, WC1N 3BG, UK. p.candler@ion.ucl.ac.uk
P M
PM
Candler
R C
RC
Dale
S
S
Griffin
A J
AJ
Church
R
R
Wait
M D
MD
Chapman
G
G
Keir
G
G
Giovannoni
J H
JH
Rees
D000293:Adolescent; D000942:Antigens, Bacterial / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D001324:Autoantigens / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D001425:Bacterial Outer Membrane Proteins / Q000276:immunology; D002462:Cell Membrane / Q000276:immunology; D002852:Chromatography, Ion Exchange / Q000379:methods; D017931:DNA Primers / Q000235:genetics; D018076:DNA, Complementary / Q000235:genetics; D004591:Electrophoresis, Polyacrylamide Gel / Q000379:methods; D005260:Female; D005956:Glucose-6-Phosphate Isomerase / Q000235:genetics / Q000276:immunology*; D006801:Humans; D015151:Immunoblotting; D007150:Immunohistochemistry; D020361:Paraneoplastic Syndromes, Nervous System / Q000175:diagnosis / Q000276:immunology* / Q000382:microbiology*; D012333:RNA, Messenger; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013290:Streptococcal Infections / Q000150:complications* / Q000276:immunology*
D000942:Antigens, Bacterial; D001323:Autoantibodies; D001324:Autoantigens; D001425:Bacterial Outer Membrane Proteins; D017931:DNA Primers; D018076:DNA, Complementary; D012333:RNA, Messenger; D005956:Glucose-6-Phosphate Isomerase