Neuroimmunology of OMS and ANNA-1/anti-Hu paraneoplastic syndromes in a child with neuroblastoma.

Neurol Neuroimmunol Neuroinflamm

National Pediatric Myoclonus Center (M.R.P., N.R.M.); and National Pediatric Neuroinflammation Organization, Inc. (M.R.P.), Orlando, FL.

Published: March 2018

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5745357PMC
http://dx.doi.org/10.1212/NXI.0000000000000433DOI Listing

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Opsoclonus myoclonus syndrome (OMS) is a rare immune-mediated paraneoplastic or para/-post-infectious syndrome characterized by "dancing" eye movements, myoclonus, and ataxia. Neuropsychiatric symptoms have also been reported. Without treatment, OMS may progress to further neurological impairment and even death.

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Absence of GluD2 Antibodies in Patients With Opsoclonus-Myoclonus Syndrome.

Neurology

February 2021

From 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.

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Opsoclonus-myoclonus syndrome (OMS) is a brainstem/cerebellar syndrome producing disabling multi-directional saccadic oscillations with oscillopsia, with or without somatic myoclonus and cerebellar ataxia (Wong et al., 2001; Armangué et al., 2016).

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