Locating neuroblastoma in the opsoclonus-myoclonus syndrome

We examined the role of various medical imaging modalities, particularly metaiodobenzylguanidine (MIBG) scintigraphy in the investigation of patients presenting with the opsoclonus-myoclonus syndrome (OMS) who may harbor neuroblastomas. A retrospective analysis was therefore performed of all patients presenting with OMS in a 5 1/2 year period. Between December, 1988 and May, 1994, all 13 patients (mean age 15.2 months, range 3 days-30 months) presenting with OMS were extensively studied. A wide range of medical imaging modalities including CT, MRI and [131I] or [123I]-metaiodobenzylguanidine (MIBG) scintigraphy (total of 21 scans) were examined as a means of detecting a structural brain lesion or locating a neuroblastoma, a tumor generally found in less than half of patients with OMS. As anticipated a minority of patients (4) were eventually found to harbor neuroblastomas. In these four cases, two tumors were revealed on preoperative MIBG scintigraphy, one gave a false negative study and one tumor was not studied preoperatively. Each patient was also subjected to extensive radiological investigations in addition to MIBG scintigraphy, many of which were repetitive, redundant or had low clinical yield. The relative merits of the various procedures are compared, and an algorithm incorporating MIBG scintigraphy and limited central nervous system and abdominal anatomical modalities for the investigation of opsoclonus-myoclonus is suggested.