OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME

  • Milan Špegel Otroški oddelek Splošna bolnišnica Gosposvetska 1 2380 Slovenj Gradec
  • Štefan Kopač Pediatrična klinika Klinični center Vrazov trg 1 1525 Ljubljana
Keywords: ataxia, opsoclonus, myoclonus, epilepsy, children, treatment, prognosis

Abstract

Background. Opsoclonus-myoclonus-ataxia syndrome (OMAS) is observed mostly in children under 3 years of age. Incidence is not known. The leading clinical sings are: opsoclonus, myoclonus and ataxia; only two signs may be present. The syndrome may follow an infectious disease usually of viral origin, but may be paraneoplastic, associated with a neuroblastoma. The course is often prolonged with recurrences but gradual and stepwise improvement. The majority of affected children are left with mental retardation, motor and/ or speech disturbances. There are no known criteria to predict prognosis. Corticosteroid or corticotropin are recommended treatment regimes.

Conclusions. The boy with OMAS is described. The diagnosis was made in retrospect. He was not treated, however inspite of this fact he has only mild mental retardation and tremor of the hands. He also developed epileptic fits which responded to antiepileptic treatment.

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How to Cite
1.
Špegel M, Kopač Štefan. OPSOCLONUS-MYOCLONUS-ATAXIA SYNDROME. TEST ZdravVestn [Internet]. 1 [cited 5Aug.2024];71(1). Available from: http://vestnik-dev.szd.si/index.php/ZdravVest/article/view/1564
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