Opsoclonus-myoclonus

David T Hsieh MD (Dr. Hsieh of the Uniformed Services University of the Health Sciences and The University of Texas Health Science Center at San Antonio has no relevant financial relationships to disclose.)
Bernard Maria MD, editor. (Dr. Maria of Icahn School of Medicine at Mount Sinai and Director of Pediatric Neurology and Developmental Medicine at Goryeb Children)
Originally released March 14, 1994; last updated October 24, 2016; expires October 24, 2019

This article includes discussion of opsoclonus-myoclonus, dancing eyes syndrome, infantile opsoclonus polymyoclonia syndrome, infantile polymyoclonus, Kinsbourne syndrome, and opsoclonus-ataxia syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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. The authors review literature on atypical sensory behaviors in children.

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 (Kinsbourne 1962), that opsoclonus-myoclonus syndrome was associated with neuroblastoma (Solomon and Chutorian 1968; Larbrisseau et al 1973; Senelick et al 1973; Berg et al 1974; Boltshauser et al 1979). 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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