Abstract
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.
References
Matthay KK, Blaes F, Hero B, Plantaz D, De Alarcon P, Mitchell WG, et al. Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer Lett. 2005;228(1):275-82.
Pranzatelli MR. The neurobiology of the opsoclonus-myoclo-nus syndrome. Clin Neuropharmacol. 1992;15(3):186-228.
Ghia T, Kanhangad M, Alessandri AJ, Price G, Gera P, Nagarajan L. Opsoclonus-myoclonus syndrome, neuroblastoma, and insulin-dependent diabetes mellitus in a child: a unique patient. Pediatr Neurol. 2016;55:68-70.
Pang KK, de Sousa C, Lang B, Pike MG. A prospective study of the presentation and management of dancing eye syndrome/ opsoclonus-myoclonus syndrome in the United Kingdom. Eur J Paediatr Neurol. 2010;14(2):156-61.
Hasegawa S, Matsushige T, Kajimoto M, Inoue H, Momonaka H, Oka M, et al. A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children. Brain & Development. 2015;37(7):656-60.
Rodríguez Rangel DA, Gelvez Pinzón JD. Síndrome opsoclonus mioclonus paraneoplásico en pediatría: reporte de caso y revisión de la literatura. Acta Neurol Colomb. 2015;31(2):209-13.
Paredes-Ebratt AM, Espinosa-García ET. Síndrome de Kins-bourne: reporte de un caso. Iatreia. 2017;30(1):81-5.
Jackson JR, Tran HC, Stein JE, Shimada H, Patel AM, Marach-elian A, et al. The clinical management and outcomes of cervical neuroblastic tumors. J Surg Res. 2016;204(1):109-13.
Wilfong AA, Parke JT, McCrary JA, 3rd. Opsoclonus-myoclo-nus with Beckwith-Wiedemann syndrome and hepatoblastoma. Pediatr Neurol. 1992;8(1):77-9.
Arroyo HA, Tringler N, De Los Santos C. Síndrome de opsoclonus-mioclonus. Medicina (B Aires). 2009;69(1 Supl.1):64-70.
Wilson LK, Draper G. Neuroblastoma, its natural history and prognosis: a study of 487 cases. Br Med J. 1974;3(5926):301-7.
Tan AH, Linn K, Sam IC, Tan CT, Lim SY. Opsoclonus-myoc-lonus-ataxia syndrome associated with dengue virus infection. Parkinsonism & Related Disorders. 2015;21(2):160-1.
Krug P, Schleiermacher G, Michon J, Valteau-Couanet D, Brisse H, Peuchmaur M, et al. Opsoclonus-myoclonus in children associated or not with neuroblastoma. Eur J Paediatr Neurol. 2010;14(5):400-9.
Pranzatelli MR, Tate ED. Trends and tenets in relapsing and progressive opsoclonus-myoclonus syndrome. Brain & Development. 2016;38(5):439-48.
Pranzatelli MR, Travelstead AL, Tate ED, Allison TJ, Verhulst SJ. CSF B-cell expansion in opsoclonus-myoclonus syndrome: A biomarker of disease activity. Mov Disord. 2004;19(7):770-7.
Pranzatelli MR, Tate ED, McGee NR, Travelstead AL, Colliver JA, Ness JM, et al. BAFF/APRIL system in pediatric OMS: relation to severity, neuroinflammation, and immunotherapy. J. Neuroinflammation. 2013;10(1):1.
Pranzatelli MR, Slev PR, Tate ED, Travelstead AL, Colliver JA, Joseph SA. Cerebrospinal fluid oligoclonal bands in childhood opsoclonus-myoclonus. Pediat Neurol. 2011;45(1):27-33.
Sivaswamy L. Approach to acute ataxia in childhood: diagnosis and evaluation. Pediatric Ann. 2014;43(4):153-9.
Haden SV, McShane MA, Holt CM. Opsoclonus myoclonus: a non-epileptic movement disorder that may present as status epilepticus. Arch Dis Child. 2009;94(11):897-9.
Zarco L, Gil L, Millán SP, Pretelt F. Síndrome de opsoc-lonus-mioclonus-ataxia (OMA) parainfeccioso secundario a infección por citomegalovirus. Acta Neurol Colomb. 2011;27(4):237-42.
Pranzatelli MR, Tate ED, Travelstead AL, Longee D. Immunologic and clinical responses to rituximab in a child with opsoc-lonus-myoclonus syndrome. Pediatrics. 2005;115(1):e115-e9.
Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K, et al. The International Neuroblastoma Risk Group (INRG) staging system: an INRG task force report. J Clin Oncol. 2009;27(2):298-303.
Lucaccioni L, Bigi E, Garcinuno CC. Incidental diagnosis of thoracic ganglioneuroblastoma in a 3 years old female with wheezing. Acta Biomed. 2012;83(1):53-5.
Takama Y, Yoneda A, Nakamura T, Nakaoka T, Higashio A, Santo K, et al. Early detection and treatment of neuroblastic tumor with opsoclonus-myoclonus syndrome improve neurological outcome: A review of five cases at a single institution in Japan. Eur J Pediatr Surg. 2016;26(1):54-9.

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