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

• Myoclonus
• Ataxia
• drooling
• refusal to walk or sit
• speech problems
• decreased muscle tone
• rage attacks
• inability to sleep

• 100% at 3 years in those presenting with opsoclonus
• 78% in those presenting with Horner syndrome
• 11% in those with orbital metastasis

• Opsoclonus typically resolves
• Cognitive and speech delays
• Motor problems: myoclonus, ataxia, dysarthria, hypotonia
• Cerebellar atrophy on later MRI

Tumor producing peptide that is neurotoxic to oculomotor system or anticerebellar antibody