Adult-onset opsoclonus-myoclonus syndrome

James P. Klaas, J. Eric Ahlskog, Sean J Pittock, Joseph Y. Matsumoto, Allen Jr. Aksamit, J. D. Bartleson, Rajeev Kumar, Kathleen F. McEvoy, Andrew B McKeon

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)1598-1607
Number of pages10
JournalArchives of Neurology
Volume69
Issue number12
DOIs
StatePublished - Dec 2012

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Opsoclonus-Myoclonus Syndrome
Immunotherapy
Onset
Syndrome
Breast
HIV

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

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Adult-onset opsoclonus-myoclonus syndrome. / Klaas, James P.; Ahlskog, J. Eric; Pittock, Sean J; Matsumoto, Joseph Y.; Aksamit, Allen Jr.; Bartleson, J. D.; Kumar, Rajeev; McEvoy, Kathleen F.; McKeon, Andrew B.

In: Archives of Neurology, Vol. 69, No. 12, 12.2012, p. 1598-1607.

Research output: Contribution to journalArticle

Klaas, JP, Ahlskog, JE, Pittock, SJ, Matsumoto, JY, Aksamit, AJ, Bartleson, JD, Kumar, R, McEvoy, KF & McKeon, AB 2012, 'Adult-onset opsoclonus-myoclonus syndrome', Archives of Neurology, vol. 69, no. 12, pp. 1598-1607. https://doi.org/10.1001/archneurol.2012.1173
Klaas JP, Ahlskog JE, Pittock SJ, Matsumoto JY, Aksamit AJ, Bartleson JD et al. Adult-onset opsoclonus-myoclonus syndrome. Archives of Neurology. 2012 Dec;69(12):1598-1607. https://doi.org/10.1001/archneurol.2012.1173
Klaas, James P. ; Ahlskog, J. Eric ; Pittock, Sean J ; Matsumoto, Joseph Y. ; Aksamit, Allen Jr. ; Bartleson, J. D. ; Kumar, Rajeev ; McEvoy, Kathleen F. ; McKeon, Andrew B. / Adult-onset opsoclonus-myoclonus syndrome. In: Archives of Neurology. 2012 ; Vol. 69, No. 12. pp. 1598-1607.
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abstract = "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.",
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AU - Klaas, James P.

AU - Ahlskog, J. Eric

AU - Pittock, Sean J

AU - Matsumoto, Joseph Y.

AU - Aksamit, Allen Jr.

AU - Bartleson, J. D.

AU - Kumar, Rajeev

AU - McEvoy, Kathleen F.

AU - McKeon, Andrew B

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N2 - 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.

AB - 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.

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