Neuromuscular DiseasesNeuromuscular DiseasesНервно-мышечные болезни2222-87212413-0443Publishing House ABV Press5410.17650/2222-8721-2013-0-3-22-26LECTURES AND REVIEWSЛЕКЦИИ И ОБЗОРЫOPSOСLONUS-MYOCLONUS SYNDROMEСиндром опсоклонуса–миоклонусаShnayderN. A.ШнайдерН. А.
Russian Federation
NASchnaider@yandex.ruKantimirovaE. A.КантимироваЕ. А.
Russian Federation
EzhikovaV. A.ЕжиковаВ. А.
Russian Federation
Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian Federation Clinical Hospital Fifty-One, Federal Biomedical Agency, ZheleznogorskГБОУ ВПО «Красноярский ГМУ им. проф. В.Ф. Войно-Ясенецкого» МЗ РФ ФГБУЗ КБ № 51 ФМБА России, ЖелезногорскProf. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian FederationГБОУ ВПО «Красноярский ГМУ им. проф. В.Ф. Войно-Ясенецкого» МЗ РФProf. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian Federation A.I. Kryzhanovsky Krasnoyarsk Territorial Cancer CenterГБОУ ВПО «Красноярский ГМУ им. проф. В.Ф. Войно-Ясенецкого» МЗ РФ КБУЗ «Красноярский краевой онкологический центр им. А.И. Крыжановского»200920133322262002201520022015Copyright ©; 2013, Shnayder N.A., Kantimirova E.A., Ezhikova V.A.Copyright ©; 2013, Шнайдер Н.А., Кантимирова Е.А., Ежикова В.А.2013Shnayder N.A., Kantimirova E.A., Ezhikova V.A.Шнайдер Н.А., Кантимирова Е.А., Ежикова В.А.https://creativecommons.org/licenses/by/4.0https://nmb.abvpress.ru/jour/article/view/54

This article provides an overview of the Russian and foreign studies on paraneoplastic opsoсlonus-myoclonus syndrome. Opsoclonus is characterized by involuntary, arrhythmic, chaotic, multi-directional saccades with horizontal, vertical and torsional components, and it is commonly accompanied by cerebellar ataxia and myoclonic jerks in the trunk and limbs. It is a rare neurological disorder of unknown causes which appears to be the result of an autoimmune process involving the nervous system. Paraneoplastic opsoсlonus-myoclonus syndrome is most commonly associated with small-cell lung cancer, breast cancer, ovarian cancer, non-Hodgkin's lymphoma, renal adenocarcinoma. In children, a neuroblastoma is detected in approximately 50% of cases. Many autoantibodies have been detected in patients with paraneoplastic opsoсlonusmyoclonussyndrome: this finding suggests the involvement of a humoral immune mechanism. However, most patients are seronegative for these autoantibodies. Paraneoplastic opsoсlonus-myoclonus syndrome is less responsive to immunotherapy (corticosteroids, intravenous immunoglobulin, adrenocorticotropic hormone, plasma exchange, cyclophosphamide, or rituximab) and improves only with tumor resection. Further studies are needed to further elucidate its immunopathogenesis and pathophysiology in order to develop novel and efficacious therapy.

paraneoplastic neurological syndromeopsoсlonus-myoclonus syndromecancerpathogenesisclinicsdiagnosistreatmentпаранеопластический неврологический синдромсиндром опсоклонуса–миоклонусаракпатогенезклиникадиагностикалечение1. Петрухин А.С., Бембеева Р.Ц., Самойлова М.В. Опсоклонус–миоклонус синдром у детей. Журн невропатол и психиатр 2006;106(2):63–6.Петрухин А.С., Бембеева Р.Ц., Самойлова М.В. Опсоклонус–миоклонус синдром у детей. Журн невропатол и психиатр 2006;106(2):63–6.2. Garg R.K., Kar A.M., Dixit V. Opsoclonus-myoclonus syndrome in an adult: A case report and response toGarg R.K., Kar A.M., Dixit V. Opsoclonus-myoclonus syndrome in an adult: A case report and response toclonazepam. Indian J Ophthalmol 1996; 44:101–2.3. Klaas J.P., Ahlskog J.E., Pittock S.J. et al. Adult-Onset Opsoclonus-Myoclonus Syndrome. Arch Neurol 2012;1–10.Klaas J.P., Ahlskog J.E., Pittock S.J. et al. Adult-Onset Opsoclonus-Myoclonus Syndrome. Arch Neurol 2012;1–10.4. Stejanowicz J., Izycka-Swieszewska E., Drozynska E. Neuroblastoma and opsoclonus – myoclonus – ataxia syndrome – clinical and pathological characteristics. Folia Neuropathol 2008;46(3):176–85.Stejanowicz J., Izycka-Swieszewska E., Drozynska E. Neuroblastoma and opsoclonus – myoclonus – ataxia syndrome – clinical and pathological characteristics. Folia Neuropathol 2008;46(3):176–85.5. Tate E., Allison Т., Pranzatelli M. Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus myoclonus syndrome. J Pediatr Oncol Nurs 2005;22(1):8–19.Tate E., Allison Т., Pranzatelli M. Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus myoclonus syndrome. J Pediatr Oncol Nurs 2005;22(1):8–19.6. Евтушенко С.К. Паранеопластические неврологические синдромы (клиника, диагностика и возможности лечения). Междунар неврол журн 2011;8(46):9–21.Евтушенко С.К. Паранеопластические неврологические синдромы (клиника, диагностика и возможности лечения). Междунар неврол журн 2011;8(46):9–21.7. Gatti G., Simsek S., Kurne A. et al. Paraneoplastic neurological disorders in breast cancer. Breast 2003;12(3):203–7.Gatti G., Simsek S., Kurne A. et al. Paraneoplastic neurological disorders in breast cancer. Breast 2003;12(3):203–7.8. Шнайдер Н.А., Дыхно Ю.А., Ежиков а В.В. Клиническая гетерогенность паранеопластического неврологического сндрома. Сиб онколог журн 2011;3(45):82–90.Шнайдер Н.А., Дыхно Ю.А., Ежиков а В.В. Клиническая гетерогенность паранеопластического неврологического сндрома. Сиб онколог журн 2011;3(45):82–90.9. Rojas-Marcos I., Rousseau A., Keime-Guibert F. et al. Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. Medicine 2003;82(3):216–23.Rojas-Marcos I., Rousseau A., Keime-Guibert F. et al. Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. Medicine 2003;82(3):216–23.10. Sa G., Correia C., Pires M. et al. Multiple paraneoplastic syndromes occurring in the same patient: clinical, imaging and neuropathological documentation. Acta Med Port 2006 ;19(6) : 489–93.Sa G., Correia C., Pires M. et al. Multiple paraneoplastic syndromes occurring in the same patient: clinical, imaging and neuropathological documentation. Acta Med Port 2006 ;19(6) : 489–93.11. Пономарев В.В. Аутоиммунные заболевания в неврологии. Минск: Беларус. навука, 2010. 259 с.Пономарев В.В. Аутоиммунные заболевания в неврологии. Минск: Беларус. навука, 2010. 259 с.12. Wong A. An update on opsoclonus. Curr Opin Neurol 2007;20(1): 25–31.Wong A. An update on opsoclonus. Curr Opin Neurol 2007;20(1): 25–31.13. Hadjivassiliou M., Boscolo S., Tongiorgi E. et al. Cerebellar ataxia as a possible organ-specific autoimmune disease. Mov Disord 2008;23:1370–7.Hadjivassiliou M., Boscolo S., Tongiorgi E. et al. Cerebellar ataxia as a possible organ-specific autoimmune disease. Mov Disord 2008;23:1370–7.14. Pranzatelli M.R. The immunopharmacology of the opsoclonusmyoclonus syndrome. Clin Neuropharmacology 1996;19(1):1–47.Pranzatelli M.R. The immunopharmacology of the opsoclonusmyoclonus syndrome. Clin Neuropharmacology 1996;19(1):1–47.15. Bataller L., Rosenfeld M.R., Graus F. et al. Autoantigen diversity in the opsoclonus-myoclonus syndrome. Ann Neurol 2003;53:347–53.Bataller L., Rosenfeld M.R., Graus F. et al. Autoantigen diversity in the opsoclonus-myoclonus syndrome. Ann Neurol 2003;53:347–53.16. Murinson B.B, Guarnaccia J.B. Stiffperson syndrome with amphiphysin antibodies: distinctive features of a rare disease. Neurology 2008;71:1955–8.Murinson B.B, Guarnaccia J.B. Stiffperson syndrome with amphiphysin antibodies: distinctive features of a rare disease. Neurology 2008;71:1955–8.17. Blaes F., Fuhlhuber V., Korfei M. et al. Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol 2005;58:313–7.Blaes F., Fuhlhuber V., Korfei M. et al. Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol 2005;58:313–7.18. Graus F., Saiz A., Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 2009;257:509–17.Graus F., Saiz A., Dalmau J. Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 2009;257:509–17.19. Vincent A., Bien C.G., Irani S.R. et al. Autoantibodies associated with diseases of the CNS: new developments and future challenges. Lancet Neurol 2011;10:759–72.Vincent A., Bien C.G., Irani S.R. et al. Autoantibodies associated with diseases of the CNS: new developments and future challenges. Lancet Neurol 2011;10:759–72.20. Vincent A., Lang B., Kleopa K.A. Autoimmune channelopathies and related neurological disorders. Neuron 2006;52:123–38.Vincent A., Lang B., Kleopa K.A. Autoimmune channelopathies and related neurological disorders. Neuron 2006;52:123–38.21. Blaes F., Pike M.G., Lang B. Autoantibodies in childhood opsoclonusmyoclonus syndrome. J Neuroimmunol 2008;201:221–6.Blaes F., Pike M.G., Lang B. Autoantibodies in childhood opsoclonusmyoclonus syndrome. J Neuroimmunol 2008;201:221–6.22. Korfei M., Fuhlhuber V., Schmidt-Woll T. et al. Functional characterisation of autoantibodies from patients with pediatric opsoclonus–myoclonus-syndrome. J Neuroimmunol 2005;170:150–7.Korfei M., Fuhlhuber V., Schmidt-Woll T. et al. Functional characterisation of autoantibodies from patients with pediatric opsoclonus–myoclonus-syndrome. J Neuroimmunol 2005;170:150–7.23. Nemni R., Braghi S., Natali-Sora M.G. et al. Autoantibodies to glutamic acid decarboxylase in palatal myoclonus and epilepsy. Ann Neurol 1994; 36:665–7.Nemni R., Braghi S., Natali-Sora M.G. et al. Autoantibodies to glutamic acid decarboxylase in palatal myoclonus and epilepsy. Ann Neurol 1994; 36:665–7.24. Kirsten A., Beck S., Fithflniber V. New autoantibodies in pediatric opsoclonus myoclonus syndrome. Ann N Y Acad Sci 2007;1110:256–60.Kirsten A., Beck S., Fithflniber V. New autoantibodies in pediatric opsoclonus myoclonus syndrome. Ann N Y Acad Sci 2007;1110:256–60.25. Sabater L., Xifro X., Saiz A. et al. Analysis of antibodies to neuronal surface antigens in adult opsoclonus-myoclonus. J Neuroimmunol 2008;196:188–91.Sabater L., Xifro X., Saiz A. et al. Analysis of antibodies to neuronal surface antigens in adult opsoclonus-myoclonus. J Neuroimmunol 2008;196:188–91.26. Blumenthal D.T., Salzman K.l., Digre K.B. et al. Early pathologic findings and long-term improvement in anti-Ma2- associated encephalitis. Neurology 2006;67:146–9.Blumenthal D.T., Salzman K.l., Digre K.B. et al. Early pathologic findings and long-term improvement in anti-Ma2- associated encephalitis. Neurology 2006;67:146–9.27. Pranzatelli M.R., Travelstead A.L., Tate E.D. et al. B- and T-cell markers in opsoclonus-myoclonus syndrome. Neurology 2004;62:1526–32.Pranzatelli M.R., Travelstead A.L., Tate E.D. et al. B- and T-cell markers in opsoclonus-myoclonus syndrome. Neurology 2004;62:1526–32.28. Pranzatelli M.R., Tate E.D., Travelstead A.L. et al. Immunologic and clinical responses to Rituximab in a child with opsoclonus-myoclonus syndrome. Pediatrics 2005;115(1):115–9.Pranzatelli M.R., Tate E.D., Travelstead A.L. et al. Immunologic and clinical responses to Rituximab in a child with opsoclonus-myoclonus syndrome. Pediatrics 2005;115(1):115–9.29. Sheela S.R., Mani P.J. Opsoclonusmyoclonus syndrome: response to plasmapheresis. Indian Pediat 2004;41:17.Sheela S.R., Mani P.J. Opsoclonusmyoclonus syndrome: response to plasmapheresis. Indian Pediat 2004;41:17.30. Bataller L., Graus F., Saiz A. et al. Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain 2001;124:437–43.Bataller L., Graus F., Saiz A. et al. Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain 2001;124:437–43.31. Pranzalelli M., Tate E., Travelstead A. Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol 2006;28(9):585–93.Pranzalelli M., Tate E., Travelstead A. Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol 2006;28(9):585–93.32. Rostasy K., Wilken В., Baumann M. High dose pulsatile dexam-ethasone therapy in children with opsoclonus myoclonus syndrome. Neuropediatrics 2006;37(5):291–5.Rostasy K., Wilken В., Baumann M. High dose pulsatile dexam-ethasone therapy in children with opsoclonus myoclonus syndrome. Neuropediatrics 2006;37(5):291–5.33. Dalmau J., Rosenfeld M.R. Paraneoplastic syndromes of the CNS. Lancet Neurol 2008;7:327–40.Dalmau J., Rosenfeld M.R. Paraneoplastic syndromes of the CNS. Lancet Neurol 2008;7:327–40.34. Giometto B., Grisold W., Vitaliani R. et al. Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. Arch Neurol 2010;67:330–5.Giometto B., Grisold W., Vitaliani R. et al. Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. Arch Neurol 2010;67:330–5.35. Graus F., Delattre J.Y., Antoine J.C. et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004;75:1135–40.Graus F., Delattre J.Y., Antoine J.C. et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004;75:1135–40.36. Zuliani L., Graus F., Giometto B. et al. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83(6):638–45.Zuliani L., Graus F., Giometto B. et al. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83(6):638–45.37. Borg M. Symptomatic myoclonus. Neurophysiol Clin 2006;36(5–6):309–18.Borg M. Symptomatic myoclonus. Neurophysiol Clin 2006;36(5–6):309–18.38. Groiss S.J., Siebler M., Schnitzler A. Full recovery of adult onset opsoclonus– myoclonus syndrome after early immunotherapy: A case report. Movement Disorders 2011;26(10):1805–7.Groiss S.J., Siebler M., Schnitzler A. Full recovery of adult onset opsoclonus– myoclonus syndrome after early immunotherapy: A case report. Movement Disorders 2011;26(10):1805–7.39. Ertle F., Behnisch W., Al Mulla N.A. et al. Treatment of neuroblastoma-related opsoclonus–myoclonus–ataxia syndrome with high-dose dexamethasone pulses. Neuroophthalmol 2012;36(4):149–52.Ertle F., Behnisch W., Al Mulla N.A. et al. Treatment of neuroblastoma-related opsoclonus–myoclonus–ataxia syndrome with high-dose dexamethasone pulses. Neuroophthalmol 2012;36(4):149–52.40. Chang B.H., Koch T., Hopkins K. et al. Neuroblastoma found in a 4-year-old after rituximab therapy for opsoclonus–myoclonus. Pediatr Blood Cancer 2008;50(3):683–7.Chang B.H., Koch T., Hopkins K. et al. Neuroblastoma found in a 4-year-old after rituximab therapy for opsoclonus–myoclonus. Pediatr Blood Cancer 2008;50(3):683–7.