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Infantile polymyoclonia-opsoclonus syndrome and neural crest tumors

From the Departments of Medicine (Division of Neurology) and Pediatrics, University of Colorado School of Medicine, Denver, Colorado

SUMMARYAn 18-month-old girl presented with incoordination and involuntary movements of acute onset. No viral etiology was proved. Cerebellar atrophy was suggested by pneumoencephalography. Opsoclonus set in later. Symptoms could be suppressed by large doses of corticotropin. Subsequently, a neural crest tumor (ganglioneuroblastoma) was suggested by chemical studies and removed surgically. After an initial three-month asymptomatic period, polymyoclonia has returned in spite of normal catecholamine secretion, but symptoms disappear on smaller doses of ACTH than previously.

Dr. Nellhaus' address is Departments of Pediatrics and Neurology, University of Colorado Medical Center, 4200 East Ninth Avenue, Denver, Colorado 80220.

This investigation was supported by training grant NB-05584 from the National Institute of Neurological Diseases and Stroke and U.S. Public Health Service grant FR69.

Submitted for publication Aug. 15, 1969; accepted Sept. 15, 1969.

The authors wish to thank Dr. James Austin for review of the manuscript, Dr. Oscar Kolar for the performance of the special immunological studies, Dr. J. Trevor Hughes of the Department of Neuropathology, Radcliffe Infirmary, United Oxford Hospitals, for his great courtesy in sending representative slides of his case (3451/64), and Dr. Stuart Schneck for his aid in the interpretation of these slides.

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