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Absence of GluD2 Antibodies in Patients With Opsoclonus-Myoclonus Syndrome
Mar Petit-Pedrol1, Mar Guasp1, Thais Armangue1
1From the Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (M.P.-P., M.G., T.A., A.S., F.G., J.D.), and Neurology Service (M.G., T.A., A.S., J.D.), Hospital Clínic, and Pediatric Neuroimmunology Unit, Department of Pediatric Neurology (T.A., C.L.), and Department of Haematology and Oncology (A.M.L.M.), Sant Joan de Déu Children Hospital, Universitat de Barcelona; Centro de Investigación Biomédica en Red Enfermedades Raras (CIBERER) (M.P.-P., M.G., T.A., J.D.), Valencia; Developmental Tumor Biology Laboratory (C.L.), Sant Joan de Déu Research Institute, Barcelona, Spain; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), Barcelona, Spain. M.P.P. is currently affiliated with the Université de Bordeaux, CNRS, Interdisciplinary Institute for Neuroscience, IINS, UMR 5297, France.
Objective:
A recent study showed glutamate receptor delta 2 antibodies (GluD2-ab) in sera of patients with opsoclonus-myoclonus syndrome (OMS). Inconsistencies between cerebellar immunoreactivity and expression of GluD2 led us to hypothesize that these antibodies are not biomarkers of OMS.
Methods:
Serum of 45 children with OMS (10 [22%] with neuroblastoma), 158 adults with OMS (53 [34%] with tumors), and 172 controls including 134 patients with several types of neurologic disorders, 18 with neuroblastoma without OMS, and 20 healthy participants were investigated. Antibodies were determined with 3 different techniques: (1) rat brain immunohistochemistry, (2) a live cell-based assay using a standard secondary antibody (2-step CBA), and (3) a similar CBA with a secondary and tertiary antibodies (3-step CBA). Two plasmids were used in the CBA studies. Three commercial GluD2-ab and 2 human sera with GluD2-ab served as controls for expression of GluD2.
Results:
The 3 commercial GluD2-ab showed predominant reactivity with the molecular and Purkinje cell layers (where GluD2 is highly enriched), and were also positive with the indicated CBAs. Substantially milder reactivity with brain tissue and CBA was obtained with the 2 control human sera containing GluD2-ab. None of the 203 patients with OMS and 172 controls showed immunoreactivities consistent with GluD2-abs. Compared with a standard 2-step CBA, the 3-step assay did not improve antibody detection and showed more frequent nonspecific reactivity that was not immunoabsorbed with GluD2.
Conclusion:
We did not find GluD2-ab in a large cohort of patients with OMS. GluD2-ab should not be considered diagnostic biomarkers of OMS.
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