1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-1-51-b 10.15844/pedneurbriefs-1-7-8 Ataxias Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Cerebellar Ataxia, Opsoclonus, and Neuroblastoma http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 12 1987 01 08 2016 1 7 51 52 Copyright: © 1987 The Author(s) 1987 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma

A 20 month-old girl with cerebellar ataxia and opsoclonus associated with neuroblastoma is reported from the Pediatric Neurology Unit, Tel Aviv Medical Center, Israel.

 Cerebellar Ataxia Neuroblastoma Ganglioneuroblastoma

A 20 month-old girl with cerebellar ataxia and opsoclonus associated with neuroblastoma is reported from the Pediatric Neurology Unit, Tel Aviv Medical Center, Israel. Ataxia, present since 1 year of age, and irregular, “jerky“ eye movements, noted on admission, became worse over a 2 month observation period. An abdominal mass found at 20 months and removed at operation was a ganglioneuroblastoma. Following surgery, steroids for 3 weeks, and chemotherapy 1 year, blood pressure returned to normal immediately and the ataxia and opsoclonus disappeared within 6-7 weeks. At a 2 year follow-up, the neurological and general examinations were normal. [1]

COMMENT. Opsomyoclonus or “dancing eye syndrome“, also known as myoclonic encephalopathy of infancy, is frequently of undetermined etiology. It may follow viral infection and it is sometimes associated with occult malignancies, notably neuroblastoma. Normal urinary catecholamines do not exclude the presence of tumor and repeat evaluations including radiographs of abdomen and chest are indicated. The acute stage of the dancing eye syndrome usually responds best to ACTH followed after a few weeks by prednisone. Steroids may need to be continued for several months.

 Harel S Yurgenson U Rechavi G Burstein Y Spirer Z Cerebellar ataxia and opsoclonus as the initial manifestations of myoclonic encephalopathy associated with neuroblastoma Childs Nerv Syst 1987 3 4 245 7 10.1007/BF00274060 3690566