Opsoclonus myoclonus syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: José Eduardo Riceto Loyola Junior, M.D.[2]

Synonyms and keywords:dancing eyes-dancing feet, dancing eye syndrome, Kinsbourne syndrome, myoclonic encephalopathy (Kinsbourne type), OMAS (opsoclonus-myoclonus-ataxia syndrome), OMS (opsoclonus myoclonus syndrome), opsoclonic encephalopathy

Overview

Opsoclonus myoclonus syndrome (OMS) is a rare neurological disorder, which can be very heterogenous, presenting itself with many different symptoms such as opsoclonus and/or myoclonus - which name the syndrome, but also ataxia, behavioral and/or sleep disturbances. It is believed to be caused by an immune system dysfunction, either induced by infection or paraneoplastic etiologies.

Historical Perspective

  • Opsoclonus myoclonus syndrome was first described in 1962 by M. Kinsbourne, who presented a series of six cases of children with ataxia, myoclonus and opsoclonus.
  • The syndrome was named as "myoclonic encephalopathy", but has also been called as "dancing eye syndrome".[1] Recently it has been more often referred to as opsoclonus myoclonus syndrome.

Classification

  • There is no established system for the classification of opsoclonus myoclonus syndrome.

Pathophysiology

Clinical Features

Differentiating Opsoclonus Myoclonus syndrome from other Diseases

Epidemiology and Demographics

  • The prevalence of opsoclonus myoclonus syndrome is approximately 1 per 1,000,000 individuals worldwide.

Age

  • Opsoclonus myoclonus syndrome is more commonly observed among patients aged 18 months old and may occur up to 5-6 years old.[6]
  • Relapses of the disease may affect adults.

Gender

  • Girls are slightly more affected with opsoclonus myoclonus syndrome than boys.[6]

Race

  • There is no racial predilection for opsoclonus myoclonus syndrome.[4]

Risk Factors

  • There are no risk factors associated with the development of opsoclonus myoclonus syndrome.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

  • The diagnosis of opsoclonus myoclonus syndrome is made when at least 3 of the following 4 diagnostic criteria are met:[8]

Symptoms

  • Opsoclonus myoclonus syndrome presents in a relapse-remitting manner.
  • There are acute and chronic symptoms.
  • The classic symptoms are mostly seen in acute disease and they are:[6]
  • The symptoms presenting in the chronic disease may be a residual opsoclonus and abnormalities of eye movement which may be elicited by re-fixating from near to far or squeezing the eyelids shut. Hypometric saccades and smooth eye pursuit movements can remain abnormal for years. Children may also remain not as coordinated as their peers. Expressive language is generally more affected than receptive language, and cognitive deficits may become more evident in teenagers.[7]

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

  • There are no other diagnostic studies that may be used to diagnose opsoclonus myoclonus syndrome.

Treatment

Medical Therapy


 
 
 
 
 
 
 
 
 
 
 
 
 
TREATMENT
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Mitigate Trigger
 
 
 
 
 
 
 
 
Treat/Retreat Neuroinflammation
 
 
 
 
 
 
 
 
 
Reassess for high risk
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Treat Infections with antimicrobials
 
Escalate or restart steroids or ACTH
 
Retest for neuroinflammation as needed
 
 
Review previous drug responses
 
Add or change modifying disease drugs
 
Formal IQ testing
 
Treat comorbid neuropsychiatric problems
 
Avoid potential pitfalls
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Give IVIG as needed
 
 
 
 
 
 
 
 
 
 
 
Select tapering method
 
 
Multimodal combination immunotherapy
 
 
 
Intensify speech therapy, PT, OT
 
 
 
 
 
 
 


Surgery

Prevention

  • There are no primary preventive measures available for opsoclonus myoclonus syndrome.

References

  1. 1.0 1.1 1.2 1.3 1.4 Blaes, Franz, and Backialakshmi Dharmalingam. "Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment." Expert review of neurotherapeutics 16.6 (2016): 641-648.
  2. Pranzatelli MR, Tate ED, McGee NR, Travelstead AL, Ranso- hoff RM, Ness JM, et al. Key role of CXCL13/CXCR5 axis for cerebrospinal fluid B cell recruitment in pediatric OMS. J Neuroimmunol 2012;243:81–8.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Pranzatelli, Michael R., and Elizabeth D. Tate. "Trends and tenets in relapsing and progressive opsoclonus-myoclonus syndrome." Brain and Development 38.5 (2016): 439-448.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 "American Academy of Ophthalmology - Opsoclonus Myoclonus Syndrome". American Academy of Ophthalmology. 07/04/2020. Check date values in: |date= (help)
  5. "ORPHANET - Opsoclonus-Myoclonus Syndrome". ORPHANET. 07/04/2020. Check date values in: |date= (help)
  6. 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 6.8 "NORD - National Organization for Rare Diseases - Opsoclonus-Myoclonus Syndrome". NORD. 07/04/2020. Check date values in: |date= (help)
  7. 7.0 7.1 7.2 7.3 7.4 7.5 7.6 7.7 Matthay, Katherine K., et al. "Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004." Cancer letters 228.1-2 (2005): 275-282.
  8. 8.0 8.1 "Genetic and Rare Diseases Information Center - Opsoclonus Myoclonus Syndrome". GARD. 07/04/2020. Check date values in: |date= (help)
  9. Pike M. Opsoclonus-myoclonus syndrome. Handb Clin Neurol 2013;112:1209–11.
  10. 10.0 10.1 "Radiopaedia - Neuroblastomas". Radiopaedia.org. 07/04/2020. Check date values in: |date= (help)
  11. 11.0 11.1 11.2 11.3 11.4 11.5 Oh, Sun-Young, Ji-Soo Kim, and Marianne Dieterich. "Update on opsoclonus–myoclonus syndrome in adults." Journal of neurology 266.6 (2019): 1541-1548.
  12. Rudnick, Emily, et al. "Opsoclonus‐myoclonus‐ataxia syndrome in neuroblastoma: Clinical outcome and antineuronal antibodies—a report from the children's cancer group study." Medical and Pediatric Oncology: The Official Journal of SIOP—International Society of Pediatric Oncology (Societé Internationale d'Oncologie Pédiatrique 36.6 (2001): 612-622.


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