Rivista di formazione e aggiornamento professionale del pediatra e del medico di base, fondata nel 1982. In collaborazione con l'Associazione Culturale Pediatri.
Login Abbonamenti Pubblicazioni Carrello Registrazione Perché registrarsi? Contatti

L'esperienza che insegna

Non cammina bene, ha “tremori” e gli occhi sembra che danzino...

A child with gait abnormalities myoclonus and dancing eyes...

Massimo Grassi1, Francesco De Leonardis1, Paola Muggeo1, Michela Sesta2, Guglielmo Paradies3, Nicola Santoro1

1UO di Pediatria “F. Vecchio”; 2UO di Neurologia Pediatrica; 3UO di Chirurgia Pediatrica Azienda Ospedaliera Universitaria Policlinico Consorziale, Ospedale Giovanni XXIII, Bari

Settembre 2014 - pagg. 445 -450

Abstract
The paper reports the case of a 15-month-old girl who presented with abnormalities of gait, myoclonus and opsoclonus. Paediatric opsoclonus-myoclonus syndrome is associated in more than 50% to a neuroblastoma, generally with favorable prognosis. Stage 2B neuroblastoma was diagnosed. The resection of the tumor, the immunosuppressive therapy and a physiotherapy rehabilitation programme have enabled to achieve a good outcome.
Contenuto riservato

Per leggere l'articolo completo è necessario effettuare il login.

Non sei ancora registrato? Registrati

Bibliografia
1. Gambini C, Conte M, Bernini G, et al. Neuroblastic tumors associated with opsoclonusmyoclonus syndrome: histological, immunoistochemical and molecular features of 15 Italian cases. Virchows Arch 2003;442:555-62. 2. Matthay KK, Blaes F, Hero B, 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; 228:275-82. 3. Rothenberg AB, Berdon WE, D’Angio GJ, Yamashiro DJ, Cowles RA. The association between neuroblastoma and opsoclonus-myoclonus syndrome: a historical review. Pediatr Radiol 2009;39:723-6. 4. Ziter FA, Bray PF, Cancilla PA. Neuropathologic findings in a patient with neuroblastoma and myoclonic encephalopathy. Arch Neurol 1979;36:51. 5. Antunes NL, Khakoo Y, Matthay KK, et al. Antineuronal antibodies in patients with neuroblastoma and paraneoplastic opsoclonusmyoclonus. J Pediatr Hematol Oncol 2000;22: 315-20. 6. Noetzel MJ, Cawley LP, James VL, Minard BJ, Agrawal HC. Anti-neurofilament protein antibodies in opsoclonus-myoclonus. J Neuroimmunol 1987;15:137-45. 7. Fisher PG, Wechsler DS, Singer HS. Anti-Hu antibody in a neuroblastoma-associated paraneoplastic syndrome. Pediatr Neurol 1994;10: 309-12. 8. Connolly AM, Pestronk A, Mehta S, Pranzatelli MR 3rd, Noetzel MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. J Pediatr 1997;130:878-84. 9. Pranzatelli MR, Travelstead AL, Tate ED, et al. B- and T-cell markers in opsoclonus-myoclonus syndrome: immunophenotyping of CSF lymphocytes. Neurology 2004;62:1526-32. 10. Fühlhuber V, Bick S, Kirsten A, et al. Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome. J Neuroimmunol 2009; 210:87-91. 11. Pranzatelli MR. The immunopharmacology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol 1996;19:1-47. 12. Pranzatelli MR. The neurobiology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol 1992;15:186-228. 13. Rudnick E, Khakoo Y, Antunes NL, et al. Opsoclonus-myoclonus ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies-a report from the Children’s Cancer Group Study. Med Pediatr Oncol 2001; 36:612-22. 14. Altman AJ, Baehner RL. Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. Cancer 1976;37:846-52. 15. Venturelli C, Guerra A, Paolucci P, Iughetti L. Dancing eye syndrome as first symptom of neuroblastoma. Acta Biomed 2013;84:162-6. 16. Koh PS, Raffensperger JG, Berry S, et al. Long-term outcome in children with opsoclonus- myoclonus and ataxia and coincident neuroblastoma. J Pediatr 1994;125:712-6. 17. Brissaud HE, Beauvais P. Opsoclonus and neuroblastoma. N Engl J Med 1969;280:1242. 18. Blake J, Fitzpatrick C. Eye signs in neuroblastoma. Trans Ophthalmol Soc UK 1972;92: 825-33. 19. Conte M. La sindrome opso-mioclonica. Hematology Meeting Report 2008;2(7):72-4. 20. Mitchell WG, Davalos-Gonzalez Y, Brumm VL, et al. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. Pediatrics 2002;109:86-98. 21. Catsman-Berrevoets CE, Aarsen FK, van Hemsbergen ML, van Noesel MM, Hakvoort- Cammel FG, van den Heuvel-Eibrink MM. Improvement of neurological status and quality of life in children with opsoclonus myoclonus syndrome at long-term follow-up. Pediatr Blood Cancer 2009;53:1048-53. 22. Hayward K, Jeremy RJ, Jenkins S, et al. Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonusmyoclonus- ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies. J Pediatr 2001;139:552-9. 23. Pohl KR, Pritchard J, Wilson J. Neurological sequelae of the dancing eye syndrome. Eur J Pediatr 1996;155:237-44. 24. Plantaz D, Michon J, Valteau-Couanet D, et al. Opsoclonus-myoclonus syndrome associated with non-metastatic neuroblastoma. Long-term survival. Study of the French Society of Pediatric Oncologists. Arch Pediatr 2000;7:621-8. 25. Gorman MP. Update on diagnosis, treatment, and prognosis in opsoclonus-myoclonus- ataxia syndrome. Curr Opin Pediatr 2010; 22(6):745-50. 26. Pranzatelli MR, Tate ED, Verhulst SJ, et al. Pediatric dosing of rituximab revisited: serum concentrations in opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol 2010;32:e167-72.

Corrispondenza: grassimassimo@hotmail.it