Opsomyoclonus

Opsomyoclonus (dancing eyes-dancing feet syndrome) is a clinical syndrome which has a strong association with neuroblastoma.

The “opso-“ refers to the presence of intermittent jerky eye movements (dancing eyes) and the “myoclonus” to myoclonic jerks and cerebellar ataxia (dancing feet).  Although only 2% of patients with neuroblastoma present with opsomyoclonus, up to 50% of patients with opsomyoclonus are ultimately found to have a neuroblastoma1.

The implication is that a child found to have opsomyoclonus must be investigated thoroughly for an otherwise occult neuroblastoma. In adults, autoimmunity secondary to infectious events rather than paraneoplastic phenomena is more common overall.

While the pathophysiology is unknown, OMS is felt to be related to autoimmunity. As a result of cerbellar Purkinje cell dysfunction, oculomotor neurons of the caudal fastigial nucleus of the cerebellum become progressively disinhibited. Anti-neuronal and anti-Purkinje cell antibodies have been implicated in OMS, however, they are not universally isolated in patients with OMD presentations 5.

A 2004 working group proposed the following clinical diagnostic criteria comprising three of the following four features 6:

  • opsoclonus
  • ataxia and/or myoclonus
  • behavioural changes or sleep disturbances
  • diagnosis of neuroblastoma

If neuroblastoma is found in a patient with opsomyoclonus, it is generally small with relatively favourable histology and the prognosis, in terms of survival, is very good. The neurologic symptoms may be treated with corticosteroids and/or ACTH, but often persist despite the medical treatment and surgical resection 2.

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Article information

rID: 12620
Synonyms or Alternate Spellings:
  • Opsoclonus
  • Opsoclonus myoclonus
  • Dancing eyes dancing feet syndrome
  • Dancing eyes syndrome (DES)

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    Neuroblastoma : presented with opsomyoclonus
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