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Opsoclonus-Myoclonus-Ataxia Syndrome

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Opsoclonus-myoclonus-ataxia syndrome (OMA) typically causes opsoclonus (conjugate, multidirectional chaotic eye movements), myoclonus, and ataxia, sometimes together with sleep disorders, cognitive deficit, and behavioral disturbance. Myoclonus is brief and spontaneous, usually with stimulus-sensitive jerks involving the limbs, palate, face, larynx, or respiratory muscles. The syndrome can develop subacutely or progress quickly.

Causes of OMA may be categorized by age of onset. In children, OMA occurs most frequently in females between 6 months and 3 years. OMA is sometimes paraneoplastic and should be suspected where it is not preceded by a definite infection. Neuroblastoma is found in more than 50% of cases.

Keywords

Purkinje Cell Cognitive Deficit Small Cell Lung Cancer Sleep Disorder Respiratory Muscle 
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.

Supplementary material

Clip 1: the patient exhibits severe, high-frequency generalized myoclonic jerks and opsoclonus. Clip 2: examination 3 months after onset shows almost complete recovery except for mild bilateral action tremor.

OMA syndrome.mp4 (MP4 39,634KB)

References

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    Pang KK, de Sousa C, Lang B. A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol. 2010;14:156–61.PubMedCrossRefGoogle Scholar
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    Bataller L, Graus F, Saiz A, et al. Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain. 2001;124:437–43.PubMedCrossRefGoogle Scholar
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    Kanjanasut N, Phanthumchinda K, Bhidayasiri R. HIV-related opsoclonus-myoclonus-ataxia syndrome: report on two cases. Clin Neurol Neurosurg. 2010;112:572–4.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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