Abstract

SILVESTRE AVENDANO, John Jairo; MUNOZ ZUNIGA, Jose Ricardo; CRUZ CALDERON, Stefania  and  MORALES VASQUEZ, Daniel Eduardo. Opsoclonus myoclonus due to ganglioneuroblastoma: case report and literature review. Acta Neurol Colomb. [online]. 2019, vol.35, n.3, pp.172-176. ISSN 0120-8748.  https://doi.org/10.22379/24224022263.

The opsoclonus myoclonus ataxia syndrome (OMA) is an infrequent entity in children, characterized opsoclonus, myoclonus/ataxia, sleep pattern or behavioral alterations. It represents great morbidity given its paraneoplastic and autoimmune nature; it is frequently associated with neuroblastic tumors and its tendency towards chronicity, relapses and neurodevelopmental sequels. We examine the case of a previously healthy thirteen months toddler, one of the earliest age reported cases in Colombia, who consulted for irritability, distal tremor, opsoclonus, and had negative neuroinfection tests. It was reported, after additional studies, the presence of 2 masses in the left pulmonary apex; one of them between the internal carotid artery and the external jugular vein. The bigger mass was surgically removed; pathology reported a ganglioneuroblastoma with nodular pattern. It was not possible to make full surgical resection and the patient experienced a relapse; as a postsurgical complication the patient had transient incomplete Horner syndrome. Due to difficult surgical access, chemotherapy was used for a whole year following the intermediate risk protocol developed by the COG (Children Oncology Group) with full resolution of the symptoms. We present the case of a toddler with difficult surgical approach where the surgical treatment failed, and complementary chemotherapy was needed. Chemotherapy turns into a therapeuthic option when surgical resection is not complete.

Keywords : ganglioneuroblastoma; infant; opsoclonus-myoclonus syndrome; paraneoplastic syndromes (MeSH).

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