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Adult-onset opsoclonus-myoclonus-ataxia syndrome as a manifestation of brazilian lyme disease-like syndrome: a case report and review of literature

Angelina Maria Martins Lino; Raphael Ribeiro Spera; Fernando Peixoto Ferraz de Campos; Christian Henrique de Andrade Freitas; Márcio Ricardo Taveira Garcia; Leonardo da Costa Lopes; Aleksander Snioka Prokopowitsch

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Described in 1962, the opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare, neurologically debilitating disorder with distinct characteristics that may begin in childhood or adult life. Although many cases remain without etiological diagnosis, others are related to neoplasms and infectious diseases. We report a 41-year-old previously healthy male with an 8-day history of headache, vertigo, nausea, vomiting, and nystagmus. After a normal brain computed tomography and lymphocytic pleocytosis in cerebral spinal fluid (CSF), intravenous acyclovir therapy was initiated in the emergency room. On the third day of hospitalization, the diagnosis of OMAS was made based on the presence of chaotic and irregular eye movements, dysarthric speech, gait instability, generalized tremor, and myoclonic jerks. In the face of his neurological worsening, ampicillin followed by nonspecific immunotherapy (methylprednisolone and intravenous immunoglobulin) was prescribed, with mild clinical improvement. After a thorough laboratory workup, the definite diagnosis of neuroborreliosis was established and ceftriaxone (4 g/daily/3 wks) and doxycycline (200 mg/day/2 mo) was administered. Toward the end of the ceftriaxone regimen, the neurologic signs substantially improved. We believe this to be the first case description of OMAS as clinical presentation of Brazilian Lyme disease-like syndrome (Baggio-Yoshinari syndrome).


Opsoclonus-Myoclonus Syndrome, Lyme Neuroborreliosis, Borrelia burgdorferi


Kinsbourne M. Myoclonic encephalopathy of infants. J Neurol N Psy. 1962;25:271-6. []

Klass JP, Ahlskog JE, Pittock SJet al. Adult-onset Opsoclonus-myoclonus Syndrome. Arch Neurol. 2012;69:1598-607. PMid: [PMID:22986354]. []

Wilken B, Baumann M, Bien CG, Hero B, Rostasy K, Hanefeld F. Chronic relapsing opsoclonus-myoclonus syndrome: Combination of cyclophosphamide and dexamethasone pulses. Eur J Paediatr Neurol. 2008;12:51-5. PMid: [PMID:17625938]. []

Cushing H, Wolbach SB. The transformation of a malignant paravertebral sympathicoblastoma into a benign ganglioneuroma. Am J Pathol. 1927;3:2003-5.

Talon P, Stoll C. Opso-myoclonus syndrome of infancy: new observations - review of the literature 110 cases. Pediàtrie. 1985;40:441-9. PMid: [PMID:3835536].

Pinsard N, Pons-Cerdan C, Mancini J, Livet MO, Bernard R. Ataxia-opsoclonus-myoclonus syndrome. Sem Hop. 1981;57:488-94. PMid: [PMID:6261394].

Rudnick E, Khakoo Y, Antunes NLet 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. PMid: [PMID:11344492]. []

Cooper R, Khakoo Y, Matthay KKet al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: histopathologic features – a report from the Children’s Cancer Group. Med Pediatr Oncol. 2001;36:623-9. PMid: [PMID:11344493]. []

Watret A, Al-Memar A. Post infective opsoclonus myoclonus ataxia syndrome (OMAS) in Adults is a treatable condition. J Neurol Neurosurg Psychiatry. 2011;82:e4. []

Ahuja R, Kumar R, Shukla R, Shukla R, Ahuja RC. Opsoclonus-myoclonus syndrome caused by varicella-zoster virus. Ann Indian Acad Neurol. 2010;13:211-2. PMid: [PMID:21085535] PMCid:PMC [PMID:21085535]. []

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;14:156-61. PMid: [PMID:19423368]. []

Wong AM1, Musallam S, Tomlinson RD, Shannon P, Sharpe JA. Opsoclonus in three dimensions: oculographics, neuropathologic and modeling correlates. J Neurol Sci. 2001;189:71-81. []

Caviness JN, Forsyth PA, Layton DD, McPhee TJ. The movement disorder of adult opsoclonus. Mov Disord. 1995;10:22-7. PMid: [PMID:7885352]. []

Digre KB. Opsoclonus in adults: report of three cases and review of literature. Arch Neurol. 1986;43:1165-75. []

Bataller L, Graus F, Saiz A, Vilchez JJ. Spanish Opsoclonus-Myoclonus Study Group: clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. Brain 2001;124(pt 2):437-43. PMid: [PMID:11157570]. []

Mitchel WG, Davalos-Gonzales Y, Brumm VLet al. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurological sequelae. Pediatrics 2001;109:86-98.

Bonnet L, Medeiros de Bustos E, Moulin T. Syndrome opsoclonus myoclonus chez l’adulte. Rev Neurol. 2012;168:987-8. PMid: [PMID:22682052]. []

Tate ED, Allinson TJ, Prazantelli MR, Verhulst SJ. Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus-myoclonus syndrome. J Pediatr Oncol Nurs. 2005;22:8-19. PMid: [PMID:15574722]. []

Brunklaus A, Pohl K, Zuberi SM, de Sousa C. Outcome and prognostic features in opsoclonus-myoclonus syndrome from infancy to adult life. Pediatrics. 2011;128:E388-94. PMid: [PMID:21788225]. []

Wirtz PW, Smitt PAES, Hoff Jet al. Anti-Ri antibody positive myoclonus in a male patient with breast carcinoma. J Neurol. 2002;249:1710-12. PMid: [PMID:12529794]. []

Berger JR, Mehari E. Paraneoplastic opsoclonus-myoclonus secondary to malignant melanoma. J Neurooncol. 1999;41:43-5. []

Jung KY, Youn J, Chung CS. Opsoclonus-myoclonus syndrome in an adult with malignant melanoma. J Neurol. 2006;253:942-3. PMid: [PMID:16715202]. []

Hauspy J, Nevin A, Harley Iet al. Paraneoplastic syndrome in vaginal melanoma: a case report and review of the literature. Int J Gynecol Cancer. 2007;17:1159-63. PMid: [PMID:17309666]. []

Scholz J, Vieregge P, Ruff C. Paraneoplastic opsoclonus-myoclonus syndrome in metastatic ovarian carcinoma. J Neurol Neurosurg Psychiatry. 1994;57:763-4. []

Jongen JL, Moll WJ, Sillevis Smitt PA, Vecht CJ, Tijssen CC. Anti-Ri positive opsoclonus-myoclonus-ataxia in ovarian duct cancer. J Neurol. 1998;245:691-2. []

Moretti R, Torre P, Antonello RM, Nasuelli D, Cazzato G. Opsoclonus-myoclonus syndrome: gabapentin as a new therapeutic proposal. Eur J Neurol. 2000;7:455-6. []

Lou E, Hensley ML, Lassman AB, Aghajanian C. Paraneoplastic opsoclonus-myoclonus syndrome secondary to immature ovarian teratoma. Gynecol Oncol. 2010;117:382-4. PMid: [PMID:20144470]. []

De Luca S, Terrone C, Crivellaro Set al. Opsoclonus-myoclonus syndrome as a paraneoplastic manifestation of renal cell carcinoma: a case report and review of the literature. Urol Int. 2002;68:206-8. []

Pittock SJ, Lucchinetti CF, Lennon VA. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments. Ann Neurol. 2003;53:580-7. PMid: [PMID:12730991]. []

Kumar A, Lajara-Nanson WA, Neilson RW Jr. Paraneoplastic opsoclonus-myoclonus syndrome: initial presentation of non-Hodgkin’s lymphoma. J Neurooncol. 2005;73:43-5. PMid: [PMID:15933816]. []

Gabaldon-Torres L, Fernandez-Domingues J, Salas-Felipe Jet al. Opsoclonus-myoclonus ataxia syndrome: two anatomo-clinical case reports. Rev Neurol. 2009;48:137-40. Spanish PMid: [PMID:19206061].

Ducrocq X, Petit J, Taillander Let al. Paraneoplastic opsoclonus-myoclonus syndrome revealing T-cell lymphoma. Presse Med. 1999;28:330-3. PMid: [PMID:10083874].

Kobayashi K, Mizukoshi C, Aoki Tet al. Borrelia burgdorferi-seropositive chronic encephalomyelopathy: Lyme neuroborreliosis? An autopsied report. Dement Geriatr Cogn Disord.1997;8:384-90. []

Peter L, Jung J, Tilikete C, Ryvlin P, Mauguiere F. Opsoclonus-myoclonus as a manifestation of Lyme disease. J Neurol Neurosurg Psychiatr. 2006;77:1090-1. PMid: [PMID:16914760] PMCid:PMC [PMID:16914760]. []

Vukelic D, Bozinovic D, Morovic M, et al. Opsoclonus-myoclonus in a child with neuroborreliosis. J Infect. 2000;40:189-91. []

Skeie GO, Eldøen G, Skeie BS, Midgard R, Kristoffersen EK, Bindoff LA. Opsoclonus-myoclonus syndrome in two cases with neuroborreliosis. Eur J Neurol. 2007;14:e1-e2. PMid: [PMID:18028183]. []

van Erp WS, Bakker NA, Aries MJH, Vroomen PCAJ. Opsoclonus and multiple cranial neuropathy as a manifestation of neuroborreliosis. Neurology. 2011;77:1013-4. PMid: [PMID:21865581]. []

Fadare O, Hart HJ. Anti-Ri antibodies associated with short term memory deficits and a mature cystic teratoma of the ovary. Int Semin Surg Oncol. 2004;1:11. PMid: [PMID:15537431] PMCid:PMC [PMID:15537431]. []

Luque FA, Furneaux HM, Ferziger Ret al. Anti-Ri: an antibody associated with paraneoplastic opsoclonus and breast cancer. Ann Neurol. 1991;29:241-51. PMid: [PMID:2042940]. []

Hersh B, Dalmau J, Dangond F, Gultekin S, Geller E, Wen PY. Paraneoplastic opsoclonus-myoclonus associated with anti-HU antibody. Neurology. 1994;44:1754-5. []

Kambe T, Yukitoshi T, Yoshiaki F. A mild form of adult-onset opsoclonus-myoclonus syndrome associated with antiglutamate receptor antibodies. JAMA Neurol. 2013;70:654-5. PMid: [PMID:23699953]. []

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. []

Noetzel M, Cawley LP, James YL, Minard BJ, Agrawal HC. Anti-neurofilament protein antibodies in opsoclonus-myoclonus. J. Neuroimmunol. 1987;15:137-45. []

Jomir L, Sirvain S, Bouly S, Garrido JF, Halle O. Syndrome opsoclonus-myoclonies: à propos d’un cas. Rev Med Interne. 2009;30:980-1. PMid: [PMID:19695745]. []

Candler PM, Dale RC, Griffin Set al. Poststreptococcal opsoclonus-myoclonus syndrome associated with antineuroleukin antibodies. J. Neurol. Neurosurg. Psychiatr. 2006;77:507-12. PMid: [PMID:16543530] PMCid:PMC [PMID:16543530]. []

Pranzatelli MR, Travelstead AL, Tate EDet al. B- and T-cell markers in opsoclonus-myoclonus syndrome. Neurology. 2004;62:1526-32. []

Pranzatelli MR, Tate ED, Travelstead ALet al. Rituximab (anti-CD-20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pedriatr Hematol Oncol. 2006;28:585-93. PMid: [PMID:17006265]. []

van Thorn R, Rabie H, Warwick JM. Opsoclons-myoclonus in an HIV-infected child on antiretroviral therapy – possible immune reconstitution inflammatory syndrome. Eur J Paediatr Neurol. 2005;9:423-6. PMid: [PMID:16269259]. []

Morita A, Ishihara M, Kamei S, Ishikawa H. Opsoclonus-Myoclonus syndrome following Influenza A infection. Intern Med. 2012;51:2429-31. []

Blue SK, Janneway R, Stanley JA. Opsoclonus and body tremulousness: a case report with suggested cause. Trans Am Neurol Assoc.1971;96:208-10. PMid: [PMID:4333997].

Mesraoua B, Abbas M, D’Souza Aet al. Adult Opsoclonus-myoclonus syndrome following Mycoplasma pneumoniae infection with dramatic response to plasmapheresis. Acta Neurol Belg. 2011;111:136-8. PMid: [PMID:21748933].

Vejjajiva A, Lerdverasirikul P. Opsoclonus in salmonella infection. Br Med J.1977;2:1260. []

Flabeau O, Meissner W, Foubert-Samier Aet al. Opsoclonus myoclonus syndrome in the context of Salmonelosis. Mov Disord. 2009;24:2306-8. PMid: [PMID:19845009]. []

Jones CE, Smyth DPL, Faust SN. Opsoclonus-myoclonus syndrome associated with group a streptococcal infection. Pediatr Infect Dis J. 2007;26:358-9. PMid: [PMID:17414405]. []

Zaganas I, Prinianakis G, Xirouchaki N, Mavridis M. Opsoclonus-myoclonus syndrome associated with cytomegalovirus encephalitis. Neurology. 2007;68:1636. PMid: [PMID:17485656]. []

Verma A, Brozman B. Opsoclonus-myoclonus syndrome following Epstein-Barr virus infection. Neurology. 2002;58:1131-2. []

Kanjanasut N, Phanthumchinda K, Bhidayasiri R. HIV-related opsoclonus-myoclonus-ataxia syndrome: report on two cases. Clin Neurol Neurosurg. 2010;112:572-4. PMid: [PMID:20427123]. []

Ertekin V, Tan H. Opsoclonus-myoclonus syndrome attributable to hepatitis C infection. Pediatr Neurol. 2000;42:441-2. PMid: [PMID:20472199]. []

Huber MB, Strozzi S, Steinlin M, Aebi C, Fluri S. Mycoplasma pneumoniae associated Opsoclonus-myoclonus syndrome in three cases. Eur J Pediatr. 2010;169:441-5. PMid: [PMID:19774394]. []

Loustalot MC, Savini H, Taugourdeau-Raymond S, Rouby F, Simon F, Jean-Pastor MJ. Opsoclonus-myoclonus syndrome in a young adult after a measles, mumps and rubella vaccine. Fundam Clin Pharmacol. 2012;26:114.

Steere AC, Malawista SE, Snydman DRet al. Lyme arthritis: an epidemic of oligoarticular arthritis in children and adults in three Connecticut communities. Arthritis Rheum. 1977;20:7-17. []

Elbaum-Garfinkle S. Close to home: a history of Yale and Lyme disease. Yale J Biol Med. 2011;84:103-8. PMid: [PMID:21698040] PMCid:PMC [PMID:21698040].

Burgdorfer W, Barbour AG, Hayes SF, Benach JL, Grunwaldt E, Davis JP. Lyme disease: a tick-borne spirochetosis? Science. 1982;216:317-9. []

Franke J, Hildebrandt A, Dorn W. Exploring gaps in our knowledge on Lyme borreliosis spirochaetes: updates on complex heterogeneity, ecology, pathogenicity. Ticks Tick Bourne Dis. 2013;4:11-25. PMid: [PMID:23246041]. []

Stanek G, Wormser GP, Gray J, Strle F. Lyme borreliosis. Lancet. 2012;379:461-73. []

Halperin JJ. Nervous system Lyme disease. J Neurol Sci. 1998;153:182-91. []

Mygland A, Ljstad U, Fingerle V, Rupprecht T, Schmutzhard, Steiner I. EFNS guidelines on the diagnosis and management of European Lyme neuroborreliosis. Eur J Neurol. 2010;17:8-16. PMid: [PMID:19930447]. []

Yoshinari NH, Mantovani E, Bonoldi VLN, Marangoni RG, Gauditano G. Brazilian Lyme-like disease or Baggio-Yoshinari Syndrome: exotic and emerging Brazilian tick-borne zoonosis. Rev Assoc Med Bras. 2010;56:363-9. Portuguese. PMid: [PMID:20676548]." xmlns:xlink="" target="_blank" title="">">"> []

Miller MM, Müllegger RR, Spork KD, Stanek G. Lyme borreliosis of central nervous system (CNS) in children: a diagnostic challenge. Infection. 1991;19:273-8. []

Djukik M, Schmidt-Samoa C, Lange Pet al. Cerebrospinal fluid findings in adults with acute Lyme neuroborreliosis. J Neurol. 2012;259:630-6. PMid: [PMID:21898139] PMCid:PMC [PMID:21898139]. []

Gouveia EA, Alves MF, Mantovani E, Oyafuso LK, Bonoldi VLN, Yoshinari NH. Profile of patients with Baggio-Yoshinari syndrome admitted at “Instituto de Infectologia Emilio Ribas”. Rev Inst Med Trop São Paulo. 2010;52:297-303. PMid: [PMID:21225212].

Shinjo SK, Gauditano G, Marchiori PEet al. Neurological manifestations in Baggio-Yoshinari syndrome (Brazilian Lyme disease – like syndrome). Bras J Rheumatol. 2009;49:492-505.

Mantovani E, Marangoni RG, Gauditano G, Bonoldi VLN, Yoshinari NH. Amplification of the flgE gene provides evidence for the existence of a Brazilian borreliosis. Rev Inst Med Trop São Paulo. 2012;54:153-7. []

Pooja D, Clarke C, David S. A case of poststreptococcal psoclonus-myoclonus syndrome. Mov Disord. 2007;22:1490-1. PMid: [PMID:17486621]. []


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