Background: Opsoclonus-myoclonus syndrome is a rare clinical condition that has been associated with neuroblastoma. There are few reported examples of ANNA-1/anti-Hu antibodies in children with neuroblastoma and opsoclonus-myoclonus, all in children aged less than three years of age.

Methods: We describe the new onset of focal seizures without alteration of consciousness and opsoclonus-myoclonus in an 11-year-old girl with ANNA-1/anti-Hu positivity and a paraspinal ganglioneuroblastoma. A systematic review of the literature of children with ANNA-1/anti-Hu positivity and malignancy was also performed.

Results: Fourteen patients were identified, eight of whom had opsoclonus-myoclonus. Although epilepsia partialis continua has been described in association with several neuronal autoantibodies, association with ANNA-1/anti-Hu has not been reported.

Conclusions: We describe epilepsia partialis continua in a child with ANNA-1/anti-Hu antibodies and neuroblastoma. Testing for antineuronal antibodies should be considered in children presenting with either opsoclonus-myoclonus or epilepsia partialis continua.