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  • Updated 12.12.2020
  • Released 03.14.1994
  • Expires For CME 12.12.2023

Opsoclonus-myoclonus

Introduction

Overview

Opsoclonus-myoclonus syndrome is a presumed autoimmune-mediated syndrome characterized by acute or subacute onset of abnormal eye movements, myoclonic jerks, ataxia, dysarthria, and behavioral changes in the setting of B-cell expansion within the cerebrospinal fluid. The etiology is most often paraneoplastic, especially neuroblastoma in children and oat cell, squamous cell, or adenocarcinoma in adults, but the etiology may also be parainfectious. After treatment for the underlying etiology, if known, medical management of neurologic symptoms usually involves immunomodulation. The long-term prognosis for many children includes some persistence of behavioral or cognitive difficulties.

Key points

• Opsoclonus-myoclonus syndrome is a presumed autoimmune-mediated syndrome characterized by acute or subacute onset of abnormal eye movements, myoclonic jerks, ataxia, dysarthria, and behavioral changes in the setting of B-cell expansion within the cerebrospinal fluid.

• The etiology is most often paraneoplastic, especially neuroblastoma in children and oat cell, squamous cell, or adenocarcinoma in adults, but the etiology may also be parainfectious.

• After surgical or medical management of the underlying etiology, if known, medical management of neurologic symptoms usually involves immunomodulation, most commonly involving ACTH, corticosteroids, or IVIg, but with an emerging use of rituximab, a monoclonal anti-B-cell antibody.

• The long-term prognosis for many children includes some persistence of behavioral or cognitive difficulties.

Historical note and terminology

The term "opsoclonus" was first used by Orzechowski in 1913 to describe rapid, chaotic, but conjugate, eye movements. It is derived from the Greek for vision (opsis) and turmoil (klonos). In 1927, Orzechowski reported the association of opsoclonus with myoclonus. However, it was not until later, following Kinsbourne's classic description of myoclonic encephalopathy in infants (47), that opsoclonus-myoclonus syndrome was associated with neuroblastoma (97; 54; 94; 09; 13). This syndrome has been given a variety of names, underscoring the difficulty in summarizing variable symptoms of opsoclonus, myoclonus, ataxia, and encephalopathy. Although "infantile myoclonic encephalopathy" and “dancing eyes-dancing feet” syndrome are terms that endure, "opsoclonus-myoclonus syndrome" better reflects current usage.

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