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Opsoclonus-Myoclonus-Ataxia Syndrome

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Part of the book series: Current Clinical Neurology ((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.

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References

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Electronic 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)

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© 2012 Springer Science+Business Media New York

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Bhidayasiri, R., Tarsy, D. (2012). Opsoclonus-Myoclonus-Ataxia Syndrome. In: Movement Disorders: A Video Atlas. Current Clinical Neurology. Humana, Totowa, NJ. https://doi.org/10.1007/978-1-60327-426-5_91

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  • DOI: https://doi.org/10.1007/978-1-60327-426-5_91

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  • Publisher Name: Humana, Totowa, NJ

  • Print ISBN: 978-1-60327-425-8

  • Online ISBN: 978-1-60327-426-5

  • eBook Packages: MedicineMedicine (R0)

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