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[Infantile myoclonic encephalopathy with opsoclonus and neuroblastoma. Apropos of 2 cases]
Insights
Infantile myoclonic encephalopathy with opsoclonus, a rare condition, can be associated with neuroblastoma. Successful treatment of the neuroblastoma led to the resolution of neurological symptoms in both reported pediatric cases.
Area of Science:
- Pediatric Oncology
- Pediatric Neurology
- Clinical Case Reports
Background:
- Infantile myoclonic encephalopathy with opsoclonus (IMEO) is a rare paraneoplastic neurological disorder.
- Neuroblastoma is a common pediatric malignancy that can present with paraneoplastic syndromes.
- The association between IMEO and neuroblastoma is uncommon but significant.
Abstract:
Two cases of infantile myoclonic encephalopathy with opsoclonus and neuroblastoma are reported. One of them was observed in a 16-month-old boy and the remaining one in a 13-month-old girl. In both cases the tumour was a stade III abdominal neuroblastoma. Urinary catecolamine excretion was increased in the girl and that of VMA in both patients. Treatment of the tumour with surgery, radiotherapy and chemotherapy resulted in a immediate disappearance of the neurologic picture in the girl. The same measures were equally successful in the boy implemented with a course of ACTH. Both children are free of tumour three years later, and they do not bear any psychomotor sequelae. The main features of this rare association are briefly commented.