Opsoclonus Myoclonus Syndrome: A Case Report

Introduction: Opsoclonus myoclonus syndrome is defined through the following criteria: opsoclonus, myoclonus, and ataxia, often behavioral and sleep problems are associated. Mostly, it is paraneoplastic associated with different kind of cancers, in children mostly with neuroblastic tumors. In discussion is an autoimmune origin.

We present videos of the course of a patient with opsoclonus myoclonus syndrome without association with a neuroblastic tumor.¹

Case Report: We report the case of an almost 3-year-old boy who developed an ataxia after an enteritis. Hospital submission was necessary by fever and persistent ataxia. Because an encephalitis was suspected antibiotic and antiviral treatment followed. Different diagnostic tools (CCT, cMRI, lumbal puncture, search for auto antibodies, and virological diagnostic) showed normal results. The next days, the boy besides the ataxia showed muscular hypotonia, sleep and behavioral problems, and an opsoclonus. A neuroblastic tumor could not be found. The therapy was started with immunoglobulins which resulted in a slightly neurologic improvement. After worsening the symptoms, a second course of immunoglobulins was given which again showed only a temporary improvement. Therefore, the decision for the treatment with corticosteroids was made. Under therapy with dexamethasone (20 mg/m²), every 4 weeks there was a good improvement. During the first four cycles, there was always a short worsening with recurrence of the opsoclonus directly before dexamethasone was given. Meanwhile there is a constant improvement.

We show videos from the first signs of opsoclonus, directly after first corticosteroid treatment up to the current state after 5 months.

Keywords: opsoclonus myoclonus neuroblastic tumor ataxia.

References

Reference

1 Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics 2013;44(6):324–329