Opsoklonus-Myoklonus-Syndrom

Introduction: Opsoclonus-myoclonus-syndrome (OMS) is a rare, idiopathic or paraneoplastic syndrome, which is predominantly characterized by opsoclonus (rapid, multi-directional conjugate eye movements), a movement disorder in terms of a myoclonic ataxia and irritability. In adulthood an association with different types of cancer has been reported, whereas in childhood neuroblastomas (NB) are found frequently.

Case report: Within a few days, a former normally developed, 13-months-old boy evolved massive ataxia, so that he was not able to stand or sit without help anymore. Further he was irritable and vomited repeatedly without other signs of infection. A cerebral MRT and the investigation of the CFS were normal. After 4 more weeks, he developed opsoclonus and myclonus of the limbs, which led to the diagnosis of OMD. An abdominal MRT revealed a lesion in the region of the left adrenal gland. The NB could be operated by laparoscopic surgery. Under therapy with dexamethoson pulses, the neurological symptoms ameliorated so far rapidly.

Conclusion: No exact data on the incidence of OMS exists, but approximately 2–3% of patients with NB develop these neurological symptoms. An autoimmune pathomechanism with cross- reactivity of an antibody to a neuroblastoma antigen and neural tissue has been postulated. The recognition of the clinical picture is for the diagnosis, search of a NB and immunmodulatoric therapies essential.