RT Journal Article SR Electronic T1 Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children JF Neurology - Neuroimmunology Neuroinflammation JO Neurol Neuroimmunol Neuroinflamm FD Lippincott Williams & Wilkins SP e1153 DO 10.1212/NXI.0000000000001153 VO 9 IS 3 A1 Thomas Rossor A1 E. Ann Yeh A1 Yasmin Khakoo A1 Paola Angelini A1 Cheryl Hemingway A1 Sarosh R. Irani A1 Gudrun Schleiermacher A1 Paramala Santosh A1 Tim Lotze A1 Russell C. Dale A1 Kumaran Deiva A1 Barbara Hero A1 Andrea Klein A1 Pedro de Alarcon A1 Mark P. Gorman A1 Wendy G. Mitchell A1 Ming Lim A1 , YR 2022 UL http://nn.neurology.org/content/9/3/e1153.abstract AB Background and Objectives Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder of the nervous system that classically presents with a combination of characteristic eye movement disorder and myoclonus, in addition to ataxia, irritability, and sleep disturbance. There is good evidence that OMAS is an immune-mediated condition that may be paraneoplastic in the context of neuroblastoma. This syndrome may be associated with long-term cognitive impairment, yet it remains unclear how this is influenced by disease course and treatment. Treatment is largely predicated on immune suppression, but there is limited evidence to indicate an optimal regimen.Methods Following an international multiprofessional workshop in 2004, a body of clinicians and scientists comprising the International OMS Study group continued to meet biennially in a joint professionals and family workshop focusing on pediatric OMAS. Seventeen years after publication of the first report, a writing group was convened to provide a clinical update on the definitions and clinical presentation of OMAS, biomarkers and the role of investigations in a child presenting with OMAS, treatment and management strategies including identification and support of long-term sequelae.Results The clinical criteria for diagnosis were reviewed, with a proposed approach to laboratory and radiologic investigation of a child presenting with possible OMAS. The evidence for an upfront vs escalating treatment regimen was reviewed, and a treatment algorithm proposed to recognize both these approaches. Importantly, recommendations on monitoring of immunotherapy response and longer-term follow-up based on an expert consensus are provided.Discussion OMAS is a rare neurologic condition that can be associated with poor cognitive outcomes. This report proposes an approach to investigation and treatment of children presenting with OMAS, based on expert international opinion recognizing the limited data available.ACTH=adrenocorticotropic hormone; ADS=acquired demyelinating syndrome; IVIg=IV immunoglobulin; MIBG=meta-iodo-benzyl-guanidine scan; OMA=opsoclonus-myoclonus-ataxia; OMAS=opsoclonus-myoclonus-ataxia syndrome; OMS=opsoclonus-myoclonus syndrome; OS=overall survival