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

Causes

  • Opsoclonus myoclonus syndrome is caused by neuroinflammation, though its mechanism is yet unknown. There is evidence supporting the neuroinflammatory theory due to the increase of oligoclonal bands on CSF. This theorized pathophysiology is discussed on its own section.

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.

Screening

  • There is insufficient evidence to recommend routine screening for opsoclonus myoclonus syndrome as it is a very rare disease.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

  • The diagnosis of opsoclonus myoclonus syndrome is made when at least 3 of the following 4 diagnostic criteria are met:[4] neuroblastoma; uncontrolled eye movement (opsoclonus); movement disorder with sudden muscle contractions (myoclonus) and/or lack of coordination (ataxia); behavioral and/or sleep disturbance.

History and 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: myoclonus, opsoclonus, ataxia.[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]
  • In children it is associated neuroblastoma in approximately half of cases.[4] In this age group it also presents with gait ataxia, dysarthria, drooling, irritability, vomiting, and insomnia.[3]
  • It has a relapse-remitting course. Symptoms may vary in duration during relapses and the remission period is also variable, but usually the relapses last at least 48-72h.[3]
  • Children affected by the disease may not be fully asymptomatic between the episodes of the disease, persisting with significant speech and language deficits, sleep and some behavioral changes.[3]
  • Most patients have no detectable antibody, but a few patients presenting with neuroblastoma do have anti-neuronal and anti-Purkinje cell antibodies.[4]
  • Children with neuroblastoma and opsoclonus myoclonus syndrome usually have a better prognosis for their neuroblastomas as they are in more differentiated stages.[1]

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with opsoclonus myoclonus syndrome.

X-ray

  • There are no specific x-ray findings associated with opsoclonus myoclonus syndrome.

Echocardiography or Ultrasound

  • There are no specific echocardiography/ultrasound findings associated with opsoclonus myoclonus syndrome.

CT scan

MRI

Other 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

Primary Prevention

  • There are no established measures for the primary prevention of opsoclonus myoclonus syndrome.

Secondary Prevention

  • Effective measures for the secondary prevention of opsoclonus myoclonus syndrome are not yet established, but chronic immunotherapy with disease modifying agents is being studied with positive results.[3]

References

  1. 1.0 1.1 1.2 1.3 1.4 Blaes F, Dharmalingam B (2016). "Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment". Expert Rev Neurother. 16 (6): 641–8. doi:10.1080/14737175.2016.1176914. PMID 27095464.
  2. Pranzatelli MR, Tate ED, McGee NR, Travelstead AL, Ransohoff RM, Ness JM; et al. (2012). "Key role of CXCL13/CXCR5 axis for cerebrospinal fluid B cell recruitment in pediatric OMS". J Neuroimmunol. 243 (1–2): 81–8. doi:10.1016/j.jneuroim.2011.12.014. PMID 22264765.
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 Pranzatelli MR, Tate ED (2016). "Trends and tenets in relapsing and progressive opsoclonus-myoclonus syndrome". Brain Dev. 38 (5): 439–48. doi:10.1016/j.braindev.2015.11.007. PMID 26786246.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 "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 6.9 "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 Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG; et al. (2005). "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 Lett. 228 (1–2): 275–82. doi:10.1016/j.canlet.2005.01.051. PMID 15922508.
  8. Pike M (2013). "Opsoclonus-myoclonus syndrome". Handb Clin Neurol. 112: 1209–11. doi:10.1016/B978-0-444-52910-7.00042-8. PMID 23622330.
  9. "Genetic and Rare Diseases Information Center - Opsoclonus Myoclonus Syndrome". GARD. 07/04/2020. Check date values in: |date= (help)
  10. Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG; et al. (2005). "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 Lett. 228 (1–2): 275–82. doi:10.1016/j.canlet.2005.01.051. PMID 15922508.
  11. 11.0 11.1 11.2 11.3 "Radiopaedia - Neuroblastomas". Radiopaedia.org. 07/04/2020. Check date values in: |date= (help)
  12. 12.0 12.1 12.2 12.3 12.4 12.5 Oh SY, Kim JS, Dieterich M (2019). "Update on opsoclonus-myoclonus syndrome in adults". J Neurol. 266 (6): 1541–1548. doi:10.1007/s00415-018-9138-7. PMID 30483882.
  13. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1038/nrdp.2016.78 Check |pmid= value (help).


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