Adult-onset opsoclonus-myoclonus syndrome.

BACKGROUND Little is known about adult-onset opsoclonus-myoclonus syndrome (OMS) outside of individual case reports. OBJECTIVE To describe adult-onset OMS. DESIGN Review of medical records (January 1, 1990, through December 31, 2011), prospective telephone surveillance, and literature review (January 1, 1967, through December 31, 2011). SETTING Department of Neurology, Mayo Clinic, Rochester, Minnesota. PATIENTS Twenty-one Mayo Clinic patients and 116 previously reported patients with adult-onset OMS. MAIN OUTCOME MEASURES Clinical course and longitudinal outcomes. RESULTS The median age at onset of the 21 OMS patients at the Mayo Clinic was 47 years (range, 27-78 years); 11 were women. Symptoms reported at the first visit included dizziness, 14 patients; balance difficulties, 14; nausea and/or vomiting, 10; vision abnormalities, 6; tremor/tremulousness, 4; and altered speech, 2. Myoclonus distribution was extremities, 15 patients; craniocervical, 8; and trunk, 4. Cancer was detected in 3 patients (breast adenocarcinoma, 2; and small cell lung carcinoma, 1); a parainfectious cause was assumed in the remainder of the patients. Follow-up of 1 month or more was available for 19 patients (median, 43 months; range, 1-187 months). Treatment (median, 6 weeks) consisted of immunotherapy and symptomatic therapy in 16 patients, immunotherapy alone for 2, and clonazepam alone for 1. Of these 19 patients, OMS remitted in 13 and improved in 3; 3 patients died (neurologic decline, 1; cancer, 1; and myocardial infarction, 1). The cause of death was of paraneoplastic origin in 60 of 116 literature review patients, with the most common carcinomas being lung (33 patients) and breast (7); the most common antibody was antineuronal nuclear antibody type 2 (anti-Ri, 15). Other causes were idiopathic in origin, 38 patients; parainfectious, 15 (human immunodeficiency virus, 7); toxic/metabolic, 2; and other autoimmune, 1. Both patients with N -methyl-D-aspartate receptor antibody had encephalopathy. Improvements were attributed to immunotherapy alone in 22 of 28 treated patients. CONCLUSIONS Adult-onset OMS is rare. Paraneoplastic and parainfectious causes (particularly human immunodeficiency virus) should be considered. Complete remission achieved with immunotherapy is the most common outcome.

[1]  J. Britton,et al.  Autoimmune epilepsy: clinical characteristics and response to immunotherapy. , 2012, Archives of neurology.

[2]  J. Parisi,et al.  Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond. , 2011, Archives of neurology.

[3]  C. Lucchinetti,et al.  N-methyl-D-aspartate receptor autoimmune encephalitis presenting with opsoclonus-myoclonus: treatment response to plasmapheresis. , 2011, Archives of neurology.

[4]  D. Zee,et al.  Opsoclonus‐myoclonus syndrome and exaggerated startle response associated with small‐cell lung cancer , 2011, Movement disorders : official journal of the Movement Disorder Society.

[5]  S. Papageorgiou,et al.  A familial case of sleep rhythmic movement disorder persistent into adulthood; approach to pathophysiology , 2011, Movement disorders : official journal of the Movement Disorder Society.

[6]  J. Bruna,et al.  Paraneoplastic opsoclonus-myoclonus syndrome as a new and single manifestation of relapsing disease in a patient with small cell lung cancer. , 2011, Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer.

[7]  I. Korn‐Lubetzki,et al.  Anxiety as initial diagnosis in opsoclonus myoclonus syndrome. , 2010, Archives of neurology.

[8]  R. Bhidayasiri,et al.  HIV-related opsoclonus–myoclonus–ataxia syndrome: Report on two cases , 2010, Clinical Neurology and Neurosurgery.

[9]  M. Sarà,et al.  Gabapentin Therapy for Ocular Opsoclonus-Myoclonus Restores Eye Movement Communication in a Patient With a Locked-In Syndrome , 2010, Neurorehabilitation and neural repair.

[10]  A. Lassman,et al.  Paraneoplastic opsoclonus-myoclonus syndrome secondary to immature ovarian teratoma. , 2010, Gynecologic oncology.

[11]  S. Bier,et al.  Emergency department presentation of a rare neurological disorder. , 2010, The Journal of emergency medicine.

[12]  E. Mowry,et al.  Movement disorders and pregnancy: A review of the literature , 2010, Movement disorders : official journal of the Movement Disorder Society.

[13]  R. Simister,et al.  Sequential fluctuating paraneoplastic ocular flutter–opsoclonus–myoclonus syndrome and Lambert–Eaton myasthenic syndrome in small-cell lung cancer , 2010, Journal of Neurology, Neurosurgery & Psychiatry.

[14]  M. Schwarz,et al.  A 76-year-old woman with acute CNS symptoms and pulmonary nodules. , 2009, Chest.

[15]  D. Guehl,et al.  Opsoclonus myoclonus syndrome in the context of Salmonellosis , 2009, Movement disorders : official journal of the Movement Disorder Society.

[16]  K. Scott,et al.  Opsoclonus–myoclonus syndrome and HIV-infection , 2009, Journal of the Neurological Sciences.

[17]  E. Foncke,et al.  False positive autoantibodies to glutamic acid decarboxylase in opsoclonus–myoclonus–ataxia syndrome after intravenous treatment with immunoglobulin , 2009, Clinical Neurology and Neurosurgery.

[18]  T. Benstead,et al.  Opsoclonus-Myoclonus with Multiple Paraneoplastic Syndromes and VGCC Antibodies , 2009, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[19]  D. Lachance,et al.  Ganglionic acetylcholine receptor autoantibody: oncological, neurological, and serological accompaniments. , 2009, Archives of neurology.

[20]  J. Taub,et al.  Insights on Chronic-Relapsing Opsoclonus-Myoclonus From a Pilot Study of Mycophenolate Mofetil , 2009, Journal of child neurology.

[21]  E. Gatto,et al.  Opsoclonus–myoclonus–ataxia syndrome and HIV seroconversion , 2009, Journal of Neurology.

[22]  A. Vincent,et al.  CLINICAL SPECTRUM OF VOLTAGE-GATED POTASSIUM CHANNEL AUTOIMMUNITY , 2009, Neurology.

[23]  X. Matías-Guiu,et al.  Anti-Ri-associated paraneoplastic cerebellar degeneration and breast cancer: An autopsy case study , 2008, Clinical Neurology and Neurosurgery.

[24]  S. Kesari,et al.  Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma , 2008, Journal of Neuro-Oncology.

[25]  A. Rombos,et al.  Opsoclonus–myoclonus–ataxia syndrome with autoantibodies to glutamic acid decarboxylase , 2008, Clinical Neurology and Neurosurgery.

[26]  B. Boeve,et al.  Clinical spectrum of voltage-gated potassium channel autoimmunity , 2008, Neurology.

[27]  K. Hassan,et al.  Long-term survival in paraneoplastic opsoclonus-myoclonus syndrome associated with small cell lung cancer. , 2008, Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society.

[28]  P. Parizel,et al.  Paraneoplastic Opsoclonus-Myoclonus-Ataxia (OMA) syndrome in an adult patient with esthesioneuroblastoma , 2008, Journal of Neurology.

[29]  L. Bindoff,et al.  Opsoclonus myoclonus syndrome in two cases with neuroborreliosis , 2007, European journal of neurology.

[30]  K. Josephs,et al.  Whole-body tremulousness: isolated generalized polymyoclonus. , 2007, Archives of neurology.

[31]  C. Clarke,et al.  A case of poststreptococcal opsoclonus‐myoclonus syndrome , 2007, Movement disorders : official journal of the Movement Disorder Society.

[32]  S. Ohara,et al.  Autopsy case of opsoclonus–myoclonus–ataxia and cerebellar cognitive affective syndrome associated with small cell carcinoma of the lung , 2007, Movement disorders : official journal of the Movement Disorder Society.

[33]  N. Xirouchaki,et al.  Opsoclonus-myoclonus syndrome associated with cytomegalovirus encephalitis , 2007, Neurology.

[34]  L. Palanivelu Chorea gravidarum , 2007, Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology.

[35]  A. Bartoš Effective High-Dose Clonazepam Treatment in Two Patients with Opsoclonus and Myoclonus: GABAergic Hypothesis , 2006, European Neurology.

[36]  W. Mason,et al.  Paraneoplastic syndrome in vaginal melanoma: a case report and review of the literature , 2006, International Journal of Gynecologic Cancer.

[37]  E. Benarroch,et al.  Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction. , 2006, Mayo Clinic proceedings.

[38]  C. Chung,et al.  Opsoclonus–myoclonus syndrome in an adult with malignant melanoma , 2006, Journal of Neurology.

[39]  A. Vincent,et al.  Anti-glial nuclear antibody: Marker of lung cancer-related paraneoplastic neurological syndromes , 2005, Journal of Neuroimmunology.

[40]  W. Lajara-Nanson,et al.  Paraneoplastic Opsoclonus-Myoclonus Syndrome: initial presentation of non-Hodgkins lymphoma , 2005, Journal of Neuro-Oncology.

[41]  M. Edelman,et al.  West Nile virus presenting as opsoclonus-myoclonus cerebellar ataxia , 2005, Neurology.

[42]  W. Leong,et al.  Anti‐Ri antibody opsoclonus‐myoclonus syndrome and breast cancer: A case report and a review of the literature , 2004, Journal of surgical oncology.

[43]  R. Koide,et al.  Opsoclonus-myoclonus syndrome during pregnancy. , 2004, Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society.

[44]  M. Gilbert,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 , 2004, Cancer investigation.

[45]  M. Kaps,et al.  Adult paraneoplastic opsoclonus-myoclonus syndrome associated with antimitochondrial autoantibodies , 2003, Journal of neurology, neurosurgery, and psychiatry.

[46]  C. Lucchinetti,et al.  Anti‐neuronal nuclear autoantibody type 2: Paraneoplastic accompaniments , 2003, Annals of neurology.

[47]  H. Meinck,et al.  Parainfectious opsoclonus-myoclonus syndrome: high dose intravenous immunoglobulins are effective , 2003, Journal of neurology, neurosurgery, and psychiatry.

[48]  J. Verschuuren,et al.  Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma , 2002, Journal of Neurology.

[49]  A. Verma,et al.  Opsoclonus-myoclonus syndrome following Epstein-Barr virus infection. , 2002, Neurology.

[50]  A. Tizzani,et al.  Opsoclonus-Myoclonus Syndrome as a Paraneoplastic Manifestation of Renal Cell Carcinoma , 2002, Urologia Internationalis.

[51]  M. Borchert,et al.  Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. , 2002, Pediatrics.

[52]  S. Gultekin,et al.  Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies. , 2001, The Journal of pediatrics.

[53]  D. Stram,et al.  Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies-a report from the Children's Cancer Group Study. , 2001, Medical and pediatric oncology.

[54]  A. Saiz,et al.  Clinical outcome in adult onset idiopathic or paraneoplastic opsoclonus-myoclonus. , 2001, Brain : a journal of neurology.

[55]  E. Benarroch,et al.  CRMP‐5 neuronal autoantibody: Marker of lung cancer and thymoma‐related autoimmunity , 2001, Annals of neurology.

[56]  R. Moretti,et al.  Opsoclonus–myoclonus syndrome: Gabapentin as a new therapeutic proposal , 2000, European journal of neurology.

[57]  P. S. Sillevis Smitt,et al.  Anti-Ri positive opsoclonus-myoclonus-ataxia in ovarian duct cancer , 1998, Journal of Neurology.

[58]  J. Caviness,et al.  Electrophysiological observations in idiopathic opsoclonus–myoclonus syndrome , 1997, Movement disorders : official journal of the Movement Disorder Society.

[59]  M. Pranzatelli The immunopharmacology of the opsoclonus-myoclonus syndrome. , 1996, Clinical neuropharmacology.

[60]  M. Pless,et al.  Treatment of opsoclonus-myoclonus with highdose intravenous immunoglobulin , 1996, Neurology.

[61]  P. O'Suilleabhain,et al.  Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. , 1995, The New England journal of medicine.

[62]  J. Caviness,et al.  The movement disorders of adult opsoclonus , 1995, Movement disorders : official journal of the Movement Disorder Society.

[63]  P. Wen,et al.  Paraneoplastic opsoclonus‐myoclonus associated with anti‐Hu antibody , 1994, Neurology.

[64]  F. Graus,et al.  Anti‐Ri antibodies associated with opsoclonus and progressive encephalomyelitis with rigidity , 1994, Neurology.

[65]  P. Vieregge,et al.  Paraneoplastic opsoclonus-myoclonus syndrome in metastatic ovarian carcinoma. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[66]  L. Kline,et al.  Antineuronal (anti‐Ri) antibodies in a patient with steroid‐responsive opsoclonus‐myoclonus , 1993, Neurology.

[67]  D. Scharf,et al.  Opsoclonus-myoclonus following the intranasal usage of cocaine. , 1989, Journal of neurology, neurosurgery, and psychiatry.

[68]  M. Rosenblum,et al.  Opsoclonus, Myoclonus, Ataxia, and Encephalopathy in Adults with Cancer: A Distinct Paraneoplastic Syndrome , 1988, Medicine.

[69]  K. Digre Opsoclonus in adults. Report of three cases and review of the literature. , 1986, Archives of neurology.

[70]  R. Payne,et al.  Paraneoplastic opsoclonus-myoclonus. Association with medullary thyroid carcinoma and review of the literature. , 1986, Archives of neurology.

[71]  A. Tamaoka,et al.  Syndrome of opsoclonus–myoclonus in hyperosmolar nonketotic coma , 1985, Annals of neurology.

[72]  N. L. Rosenberg Hearing loss as an initial symptom of the opsoclonus-myoclonus syndrome. , 1984, Archives of neurology.

[73]  C. Cordon-Cardo,et al.  OPSOCLONUS AND OAT CELL CARCINOMA OF LUNG: LACK OF EVIDENCE FOR ANTI-CNS ANTIBODIES , 1984, The Lancet.

[74]  N. K. Singh,et al.  Loperamide versus copamide. , 1984, The Journal of the Association of Physicians of India.

[75]  J. Gawler,et al.  Opsoclonus and polymyoclonia complicating oat-cell carcinoma of the bronchus , 1982, Postgraduate medical journal.

[76]  B. Jabbari,et al.  Abnormal visual-evoked responses and opsoclonus. , 1981, Journal of clinical neuro-ophthalmology.

[77]  C. L. Lim,et al.  Electro‐oculographic analysis of opsoclonus , 1977, Neurology.

[78]  M Swash,et al.  Progressive encephalomyelitis with rigidity. , 1976, Brain : a journal of neurology.

[79]  H. Boddie Ocular bobbing and opsoclonus Two abnormal spontaneous eye movements occurring in the same patient: case report , 2022 .

[80]  M. Wéber,et al.  EMG study of opsoclonus occurring in vertebro-basilar insufficiency. , 1970, Electroencephalography and clinical neurophysiology.

[81]  D. McLean Polymyoclonia with opsoclonus , 1970, Neurology.

[82]  V. P. Sweeney,et al.  An acute syndrome of ocular oscillations and truncal myoclonus. , 1968, Brain : a journal of neurology.

[83]  D. Cogan Opsoclonus, body tremulousness, and benign encephalitis. , 1968, Archives of ophthalmology.

[84]  A. T. Ross,et al.  Opsoclonus, occult carcinoma, and chemical pathology in dentate nuclei. , 1967, Transactions of the American Neurological Association.

[85]  M. Kinsbourne Myoclonic encephalopathy of infants , 1962, Journal of neurology, neurosurgery, and psychiatry.

[86]  J. Dalmau,et al.  Opsoclonus-myoclonus syndrome in anti-N-methyl-D-aspartate receptor encephalitis. , 2010, Archives of neurology.

[87]  J. Berger,et al.  Paraneoplastic Opsoclonus–myoclonus Secondary to Malignant Melanoma , 2004, Journal of Neuro-Oncology.

[88]  G. Mann,et al.  Acute cerebellar ataxia with opsoclonus. , 1984, The Journal of the Association of Physicians of India.