Opsoclonus myoclonus ataxia

INTRODUCTION

Opsoclonus myoclonus ataxia (OMA) is a syndrome that includes opsoclonus along with diffuse or focal body myoclonus and truncal titubation with or without ataxia and other cerebellar signs.

  • Opsoclonus is a disorder of ocular motility characterized by spontaneous, arrhythmic, conjugate saccades occurring in all directions of gaze without a saccadic interval.
  • Myoclonus is a clinical sign that is characterized by brief, shock-like, involuntary movements caused by muscular contractions or inhibitions.

Patients with OMA are sometimes described as having dancing eyes and feet. Although OMA can be paraneoplastic in origin, it can also result from viral infections, post-streptococcal pharyngitis, metabolic disorders, metastases, and intracranial hemorrhage [1,2].

This topic discusses opsoclonus myoclonus ataxia. An overview of paraneoplastic syndromes and other paraneoplastic disorders are discussed separately. Other disorders causing myoclonus are also discussed separately. (See "Overview of paraneoplastic syndromes of the nervous system" and "Paraneoplastic syndromes affecting peripheral nerve and muscle" and "Paraneoplastic syndromes affecting the spinal cord and dorsal root ganglia" and "Classification and evaluation of myoclonus" and "Symptomatic (secondary) myoclonus" and "Treatment of myoclonus".)

PEDIATRIC SYNDROME

OMA is rare; one prospective study in the United Kingdom estimated an incidence of 0.18 cases per million per year [3]. OMA affects mainly young children with a mean age of 1.5 to 2 years [3,4]. Some series, but not others, have found a higher prevalence in girls. A female predominance is characteristic of most, but not all, autoimmune disorders [5].  

            

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Literature review current through: Jun 2014. | This topic last updated: Jul 16, 2013.
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References
Top
  1. Digre KB. Opsoclonus in adults. Report of three cases and review of the literature. Arch Neurol 1986; 43:1165.
  2. Candler PM, Dale RC, Griffin S, et al. Post-streptococcal opsoclonus-myoclonus syndrome associated with anti-neuroleukin antibodies. J Neurol Neurosurg Psychiatry 2006; 77:507.
  3. 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; 14:156.
  4. Tate ED, Allison TJ, Pranzatelli MR, Verhulst SJ. Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus-myoclonus syndrome. J Pediatr Oncol Nurs 2005; 22:8.
  5. Nussinovitch U, Shoenfeld Y. The role of gender and organ specific autoimmunity. Autoimmun Rev 2012; 11:A377.
  6. 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.
  7. Connolly AM, Pestronk A, Mehta S, et al. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues. J Pediatr 1997; 130:878.
  8. Blaes F, Fühlhuber V, Korfei M, et al. Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol 2005; 58:313.
  9. Korfei M, Fühlhuber V, Schmidt-Wöll T, et al. Functional characterisation of autoantibodies from patients with pediatric opsoclonus-myoclonus-syndrome. J Neuroimmunol 2005; 170:150.
  10. Pranzatelli MR, Tate ED, McGee NR, Ransohoff RM. CCR7 signaling in pediatric opsoclonus-myoclonus: upregulated serum CCL21 expression is steroid-responsive. Cytokine 2013; 64:331.
  11. Krasenbrink I, Fühlhuber V, Juhasz-Boess I, et al. Increased prevalence of autoimmune disorders and autoantibodies in parents of children with opsoclonus-myoclonus syndrome (OMS). Neuropediatrics 2007; 38:114.
  12. 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.
  13. Antunes NL, Khakoo Y, Matthay KK, et al. Antineuronal antibodies in patients with neuroblastoma and paraneoplastic opsoclonus-myoclonus. J Pediatr Hematol Oncol 2000; 22:315.
  14. Ko MW, Dalmau J, Galetta SL. Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol 2008; 28:58.
  15. Ertekin V, Tan H. Opsoclonus-myoclonus syndrome attributable to hepatitis C infection. Pediatr Neurol 2010; 42:441.
  16. Vukelic D, Bozinovic D, Morovic M, et al. Opsoclonus-myoclonus syndrome in a child with neuroborreliosis. J Infect 2000; 40:189.
  17. Cardesa-Salzmann TM, Mora J, García Cazorla MA, et al. Epstein-Barr virus related opsoclonus-myoclonus-ataxia does not rule out the presence of occult neuroblastic tumors. Pediatr Blood Cancer 2006; 47:964.
  18. Sheth RD, Horwitz SJ, Aronoff S, et al. Opsoclonus myoclonus syndrome secondary to Epstein-Barr virus infection. J Child Neurol 1995; 10:297.
  19. van Toorn R, Rabie H, Warwick JM. Opsoclonus-myoclonus in an HIV-infected child on antiretroviral therapy--possible immune reconstitution inflammatory syndrome. Eur J Paediatr Neurol 2005; 9:423.
  20. Kuban KC, Ephros MA, Freeman RL, et al. Syndrome of opsoclonus-myoclonus caused by Coxsackie B3 infection. Ann Neurol 1983; 13:69.
  21. Huber BM, Strozzi S, Steinlin M, et al. Mycoplasma pneumoniae associated opsoclonus-myoclonus syndrome in three cases. Eur J Pediatr 2010; 169:441.
  22. Deconinck N, Scaillon M, Segers V, et al. Opsoclonus-myoclonus associated with celiac disease. Pediatr Neurol 2006; 34:312.
  23. 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.
  24. Wells EM, Dalmau J. Paraneoplastic neurologic disorders in children. Curr Neurol Neurosci Rep 2011; 11:187.
  25. Wong A. An update on opsoclonus. Curr Opin Neurol 2007; 20:25.
  26. Matsumura K, Sonoh M, Tamaoka A, Sakuta M. Syndrome of opsoclonus-myoclonus in hyperosmolar nonketotic coma. Ann Neurol 1985; 18:623.
  27. Desai J, Mitchell WG. Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome. J Child Neurol 2012; 27:1482.
  28. Pranzatelli MR, Tate ED, Wheeler A, et al. Screening for autoantibodies in children with opsoclonus-myoclonus-ataxia. Pediatr Neurol 2002; 27:384.
  29. Hayward K, Jeremy RJ, Jenkins S, et al. Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies. J Pediatr 2001; 139:552.
  30. Hammer MS, Larsen MB, Stack CV. Outcome of children with opsoclonus-myoclonus regardless of etiology. Pediatr Neurol 1995; 13:21.
  31. Mitchell WG, Snodgrass SR. Opsoclonus-ataxia due to childhood neural crest tumors: a chronic neurologic syndrome. J Child Neurol 1990; 5:153.
  32. Russo C, Cohn SL, Petruzzi MJ, de Alarcon PA. Long-term neurologic outcome in children with opsoclonus-myoclonus associated with neuroblastoma: a report from the Pediatric Oncology Group. Med Pediatr Oncol 1997; 28:284.
  33. Pranzatelli MR, Tate ED, Kinsbourne M, et al. Forty-one year follow-up of childhood-onset opsoclonus-myoclonus-ataxia: cerebellar atrophy, multiphasic relapses, and response to IVIG. Mov Disord 2002; 17:1387.
  34. Rostásy K, Wilken B, Baumann M, et al. High dose pulsatile dexamethasone therapy in children with opsoclonus-myoclonus syndrome. Neuropediatrics 2006; 37:291.
  35. Wilken B, Baumann M, Bien CG, et al. Chronic relapsing opsoclonus-myoclonus syndrome: combination of cyclophosphamide and dexamethasone pulses. Eur J Paediatr Neurol 2008; 12:51.
  36. Klein A, Schmitt B, Boltshauser E. Long-term outcome of ten children with opsoclonus-myoclonus syndrome. Eur J Pediatr 2007; 166:359.
  37. Pranzatelli MR, Tate ED, Travelstead AL, et al. Insights on chronic-relapsing opsoclonus-myoclonus from a pilot study of mycophenolate mofetil. J Child Neurol 2009; 24:316.
  38. Ertle F, Behnisch W, Al Mulla NA, et al. Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses. Pediatr Blood Cancer 2008; 50:683.
  39. Pranzatelli MR, Tate ED, Travelstead AL, et al. Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol 2006; 28:585.
  40. Bell J, Moran C, Blatt J. Response to rituximab in a child with neuroblastoma and opsoclonus-myoclonus. Pediatr Blood Cancer 2008; 50:370.
  41. Tate ED, Pranzatelli MR, Verhulst SJ, et al. Active comparator-controlled, rater-blinded study of corticotropin-based immunotherapies for opsoclonus-myoclonus syndrome. J Child Neurol 2012; 27:875.
  42. De Grandis E, Parodi S, Conte M, et al. Long-term follow-up of neuroblastoma-associated opsoclonus-myoclonus-ataxia syndrome. Neuropediatrics 2009; 40:103.
  43. Mitchell WG, Davalos-Gonzalez Y, Brumm VL, et al. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. Pediatrics 2002; 109:86.
  44. Krug P, Schleiermacher G, Michon J, et al. Opsoclonus-myoclonus in children associated or not with neuroblastoma. Eur J Paediatr Neurol 2010; 14:400.
  45. Catsman-Berrevoets CE, Aarsen FK, van Hemsbergen ML, et al. 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.
  46. Pranzatelli MR, Tate ED, Dukart WS, et al. Sleep disturbance and rage attacks in opsoclonus-myoclonus syndrome: response to trazodone. J Pediatr 2005; 147:372.
  47. Bataller L, Graus F, Saiz A, et al. Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain 2001; 124:437.
  48. Klaas JP, Ahlskog JE, Pittock SJ, et al. Adult-onset opsoclonus-myoclonus syndrome. Arch Neurol 2012; 69:1598.
  49. Bataller L, Rosenfeld MR, Graus F, et al. Autoantigen diversity in the opsoclonus-myoclonus syndrome. Ann Neurol 2003; 53:347.
  50. Luque FA, Furneaux HM, Ferziger R, et al. Anti-Ri: an antibody associated with paraneoplastic opsoclonus and breast cancer. Ann Neurol 1991; 29:241.
  51. Buckanovich RJ, Yang YY, Darnell RB. The onconeural antigen Nova-1 is a neuron-specific RNA-binding protein, the activity of which is inhibited by paraneoplastic antibodies. J Neurosci 1996; 16:1114.
  52. Pittock SJ, Lucchinetti CF, Lennon VA. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments. Ann Neurol 2003; 53:580.
  53. Fitzpatrick AS, Gray OM, McConville J, McDonnell GV. Opsoclonus-myoclonus syndrome associated with benign ovarian teratoma. Neurology 2008; 70:1292.
  54. Jung KY, Youn J, Chung CS. Opsoclonus-myoclonus syndrome in an adult with malignant melanoma. J Neurol 2006; 253:942.
  55. Kawachi I, Saji E, Toyoshima Y, et al. Treatment responsive opsoclonus-ataxia associated with ovarian teratoma. J Neurol Neurosurg Psychiatry 2010; 81:581.
  56. Erlich R, Morrison C, Kim B, et al. ANNA-2: an antibody associated with paraneoplastic opsoclonus in a patient with large-cell carcinoma of the lung with neuroendocrine features--correlation of clinical improvement with tumor response. Cancer Invest 2004; 22:257.
  57. Dropcho EJ, Kline LB, Riser J. Antineuronal (anti-Ri) antibodies in a patient with steroid-responsive opsoclonus-myoclonus. Neurology 1993; 43:207.
  58. Lewis MA, Hartmann LC, Lachance DH, Jimenez RE. Opsoclonus as a suspected paraneoplastic syndrome of endometrial cancer. Rare Tumors 2010; 2:e42.
  59. Weizman DA, Leong WL. Anti-Ri antibody opsoclonus-myoclonus syndrome and breast cancer: a case report and a review of the literature. J Surg Oncol 2004; 87:143.
  60. Yang YY, Yin GL, Darnell RB. The neuronal RNA-binding protein Nova-2 is implicated as the autoantigen targeted in POMA patients with dementia. Proc Natl Acad Sci U S A 1998; 95:13254.
  61. Peter L, Jung J, Tilikete C, et al. Opsoclonus-myoclonus as a manifestation of Lyme disease. J Neurol Neurosurg Psychiatry 2006; 77:1090.
  62. Wiest G, Safoschnik G, Schnaberth G, Mueller C. Ocular flutter and truncal ataxia may be associated with enterovirus infection. J Neurol 1997; 244:288.
  63. Alshekhlee A, Sultan B, Chandar K. Opsoclonus persisting during sleep in West Nile encephalitis. Arch Neurol 2006; 63:1324.
  64. Verma A, Brozman B. Opsoclonus-myoclonus syndrome following Epstein-Barr virus infection. Neurology 2002; 58:1131.
  65. Kanjanasut N, Phanthumchinda K, Bhidayasiri R. HIV-related opsoclonus-myoclonus-ataxia syndrome: report on two cases. Clin Neurol Neurosurg 2010; 112:572.
  66. Scott KM, Parker F, Heckmann JM. Opsoclonus-myoclonus syndrome and HIV-infection. J Neurol Sci 2009; 284:192.
  67. Ayarza A, Parisi V, Altclas J, et al. Opsoclonus-myoclonus-ataxia syndrome and HIV seroconversion. J Neurol 2009; 256:1024.
  68. Flabeau O, Meissner W, Foubert-Samier A, et al. Opsoclonus myoclonus syndrome in the context of Salmonellosis. Mov Disord 2009; 24:2306.
  69. Zaganas I, Prinianakis G, Xirouchaki N, Mavridis M. Opsoclonus-myoclonus syndrome associated with cytomegalovirus encephalitis. Neurology 2007; 68:1636.
  70. Lapenna F, Lochi L, Iliceto G, et al. Post-vaccinic opsoclonus-myoclonus syndrome: a case report. Parkinsonism Relat Disord 2000; 6:241.
  71. Dassan P, Clarke C, Sharp DJ. A case of poststreptococcal opsoclonus-myoclonus syndrome. Mov Disord 2007; 22:1490.
  72. Anderson NE, Budde-Steffen C, Rosenblum MK, et al. Opsoclonus, myoclonus, ataxia, and encephalopathy in adults with cancer: a distinct paraneoplastic syndrome. Medicine (Baltimore) 1988; 67:100.
  73. Hormigo A, Dalmau J, Rosenblum MK, et al. Immunological and pathological study of anti-Ri-associated encephalopathy. Ann Neurol 1994; 36:896.
  74. Armangue T, Titulaer MJ, Sabater L, et al. A novel treatment-responsive encephalitis with frequent opsoclonus and teratoma. Ann Neurol 2014; 75:435.
  75. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol 2008; 7:327.
  76. Cher LM, Hochberg FH, Teruya J, et al. Therapy for paraneoplastic neurologic syndromes in six patients with protein A column immunoadsorption. Cancer 1995; 75:1678.
  77. Bartos A. Effective high-dose clonazepam treatment in two patients with opsoclonus and myoclonus: GABAergic hypothesis. Eur Neurol 2006; 56:240.
  78. Fernandes TD, Bazan R, Betting LE, da Rocha FC. Topiramate effect in opsoclonus-myoclonus-ataxia syndrome. Arch Neurol 2012; 69:133.