Neuroblastoma
- 2-3% of patients with neuroblastoma have opsoclonus-myoclonus-ataxia syndrome
- 50% of those with opsoclonus have neuroblastoma
- Children with or without tumors cannot be distinguished by neurologic symptoms
Associated symptoms
- Myoclonus
- Ataxia
- drooling
- refusal to walk or sit
- speech problems
- decreased muscle tone
- rage attacks
- inability to sleep
Survival
- 100% at 3 years in those presenting with opsoclonus
- 78% in those presenting with Horner syndrome
- 11% in those with orbital metastasis
Late problems
- Opsoclonus typically resolves
- Cognitive and speech delays
- Motor problems: myoclonus, ataxia, dysarthria, hypotonia
- Cerebellar atrophy on later MRI
Etiology
Tumor producing peptide that is neurotoxic to oculomotor system or anticerebellar antibody