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Review
. 2022 Feb;269(2):750-757.
doi: 10.1007/s00415-021-10536-3. Epub 2021 Mar 29.

Opsoclonus-myoclonus-ataxia syndrome in children

Poonam Bhatia  1   2 , Jennifer Heim  1 , Patricia Cornejo  2 , Lauren Kane  1   3 , Jason Santiago  1 , Michael C Kruer  4   5   6
Affiliations
Review

Opsoclonus-myoclonus-ataxia syndrome in children

Poonam Bhatia et al. J Neurol. 2022 Feb.

Abstract

Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.

Keywords: Movement disorders; Neuroimmunology; Opsoclonus–myoclonus–ataxia; Pediatric.

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