Clinical Feature of the Dancing Eye Syndrome / 대한소아신경학회지

ABSTRACT

PURPOSE: The dancing eye syndrome is a rare neurological condition of unknown etiology characterized by multidirectional chaotic eye movement(opsoclonus), myoclons and ataxia. In children, it could be a paraneoplastic syndrome in association with neuroblastoma. Long-term neurological sequelae and decreased visual acuity are major problem in these patients. This study was done retrospectively to evaluate the clinical course and neurologic sequelae of the dancing eye syndrome and we also investigated in association with neuroblastoma. METHODS: We retrospectively reviewed the medical records of 5 childrens who were admitted to Pediatric Neurology, College of Medicine, Yonsei University for dancing eye syndrome from 1990 to 2001. RESULTS: The range of age was from 4 months to 2 years 9 months, 4 of them were female and the rest one was male. The mean follow up duration was 4 years 6 months. The associated diseases were hypoxic ischemic encephalopathy(2 cases), congenital aniridia(1 case). Neuroblastoma was identified in only 1 children, stage 2A. Four of them had recurrence of opsoclonus and visual acuity were getting worse(near blindness in 1 case). Four patients had a long-term neurological sequelae. The long-term neurological sequelae was dysarthria, learning disorder, seizure, ataxia. CONCLUSION: MIBG(Metaiodobenzylguanidine) scan and abdominal sonography are highly effective in the detection of neuroblastoma. Steroid therapy seems to be effective in opsoclonus in acute stage but did not necessarily have a good long term neurological outcome as it recurred. In conclusion, major problem in dancing eye syndrome is not opsoclonus and myoclonus in acute stage but decreased visual acuity and long-term neurological sequelae.