Opsoclonus-Myoclonus Syndrome in Childhood

  • F. Blaes
  • B. Lang
Part of the Topics in Neuroscience book series (TOPNEURO)


In 1962 Kinsbourne described six infants with a syndrome of myoclonus and “dancing eyes” (opsoclonus-myoclonus syndrome, OMS) [1]. OMS in association with neuroblastoma [2] and other neural-crest-derived tumours has been described in some children (paraneoplastic OMS, OMS-NB+), whilst several other potential causal relationships have also been found (Table 1), but little progress has been made in understanding the etiology and treatment of this syndrome. Recently, however, there has been increasing evidence that OMS may be an autoimmune disease of the central nervous system. This chapter will describe the clinical and immunological features as well as treatment options of OMS.
Table 1

Neurological diseases characterized by epilepsy and autoantibodies


Antibody Target


Rasmussen’s encephalitis (RE) Epilepsia partialis continua Drug-resistant


Plasma exchange or protein A immunoadsorption

Systemic lupus erythematosus (SLE) Primary generalized before SLE onset

Phospholipid, Cardiolipin β2-glycoprotein I

Not reported

Therapy-resistant localization-related epilepsy

Cardiolipin, nuclear, β2-glycoprotein I GADa

Not reported

Newly diagnosed seizure

Cardiolipin, nuclear β2-glycoprotein I

Not reported

Generalized epilepsy syndromes


Not reported

West’s syndrome


Corticosteroids, intravenuous therapy

Cryptogenic Lennox-Gastaut syndrome


Intravenous therapy

Completely-controlled epilepsy


Not reported

a The presence of anti-GAD antibodies in uncontrolled and completely-controlled epilepsy is disputed [56, 57]


Paraneoplastic Neurological Syndrome Ibotenic Acid Pediatric Oncology Group Potential Causal Relationship Omnipause Neuron 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Italia 2002

Authors and Affiliations

  • F. Blaes
    • 1
  • B. Lang
    • 2
  1. 1.Department of NeurologyJustus-Liebig UniversityGiessenGermany
  2. 2.Neurosciences GroupWeatherall Institute of Molecular Medicine, John Radcliffe HospitalOxfordUK

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