Opsoclonus-myoclonus syndrome usually sets in abruptly and can become chronic; remission and relapses have also been described, with motor, cognitive and behavioral impairment. The disease usually affects children between 1 and 3 years of age, but adult onset has also been reported. The ataxia can appear in a subacute manner, over several weeks, making the diagnosis more difficult, as opsoclonus usually appears later.

Symptoms consist of opsoclonus, myoclonus, ataxia, irritability, dysarthria or mutism, hypotonia, vomiting [1], head tilt, excessive drooling, malaise, incoordination, cranial nerve palsies, seizures and sleep disturbances [2]. Encephalopathy manifests as slow ideation and mood changes.

Opsoclonus is characterized by spontaneous, arrhythmic, conjugate saccades of the eyes, that might initially manifest as ocular flutter. Symptoms persist during sleep.

Myoclonus represents involuntary, brief movements caused by muscular contractions or inhibitions, involving the face, eyelids, limbs, fingers, head and trunk. It may make sitting or standing impossible and becomes worse when the patient is agitated.

Patients also present with behavior disturbances such as rage attacks, irritability or apathy, and sleep pattern changes.

Opsoclonus-myoclonus syndrome is usually associated with neuroblastoma in children [2] and small-cell lung cancer or breast adenocarcinoma in adults. Other conditions that should be excluded are melanoma [3], gynecologic malignancies [4], urologic cancers [5], hematologic [6] and gastrointestinal neoplasms.

• Disability

  A severe initial presentation in 82% patients predicted a chronic course and later learning disability. Cognitive impairment occurred in patients younger at disease onset. [pediatricneurologybriefs.com]

  A severe initial presentation predicted a chronic disease course (odds ratio [OR]: 2.77 [95% confidence interval (CI): 1.47-5.23]; P = .002) and later learning disability (OR: 2.03 [95% CI: 1.08-3.79]; P = .026). [ncbi.nlm.nih.gov]

  A severe initial presentation predicted a chronic disease course (odds ratio [OR]: 2.77 [95% confidence interval (CI): 1.47–5.23]; P = .002) and later learning disability (OR: 2.03 [95% CI: 1.08–3.79]; P = .026). [pediatrics.aappublications.org]

• Malaise

  Disease course and clinical subtypes In most cases OMS starts with an acute flare-up of physical symptoms within days or weeks, but some less obvious symptoms such as irritability and malaise may begin weeks or months earlier. [bionity.com]

  Symptoms consist of opsoclonus, myoclonus, ataxia, irritability, dysarthria or mutism, hypotonia, vomiting, head tilt, excessive drooling, malaise, incoordination, cranial nerve palsies, seizures and sleep disturbances. [symptoma.com]

  […] comprehension of speech, caused by brain damage) mutism (a language disorder in which a person does not speak despite evidence of speech ability in the past, often part of a larger neurological or psychiatric disorder) lethargy[4][5] irritability or malaise [en.wikipedia.org]

• Fatigue

  Injuries compensated by the Vaccine Injury Compensation Program include the following: Allergic Reaction/Anaphylaxis Autoimmune Disorders Blood Clots Blood Disorders Bowel Injury Brain Damage Chronic Fatigue Death Nerve Damage [vaccineinjuryhelpcenter.com]

  The symptoms may include upper respiratory tract infections, gastroenteritis, fever, fatigue, or confusion.2-4 The most common type of seizure is focal motor that can transition into tonic-clinic seizures. [consultant360.com]

  Review of Systems Positive for recent upper respiratory illness symptoms, fatigue, night sweats, nausea, and vomiting. Negative for acute decrease in vision. [eyerounds.org]

  He developed increasing fatigue and dyspnea but denied wheezing or chest tightness. He had watery, non-bloody diarrhea throughout the febrile period until his cough improved, and he became afebrile 11 days after diagnosis. [tremorjournal.org]

  These can include: Dark circles around the eyes Bulging eyes Bone pain Swollen belly, or the feeling of a painless lump in the belly Fever Weight loss Easy bruising or bleeding High blood pressure Fatigue CAUSES Neuroblastoma A neuroblastoma is a type [childneurologyfoundation.org]

• Episodic Course

  Onset of a specific episode typically can be assigned to a particular day or week, at which time symptoms seem to "explode" in severity, and they are frequently associated with an infectious episode. (4) There must be evidence of an antecedent or concomitant [webpediatrics.com]

• Myoclonus

  Myoclonus indicates sudden, quick jerks of a muscle. In children this condition is often associated with neuroblastoma. Neuroblastoma in a child Most adults with opsoclonus myoclonus have neoplastic, infectious, metabolic, or idiopathic etiologies. [pathology-india.com]

  Diagnosis - Opsoclonus Myoclonus Syndrome Prognosis - Opsoclonus Myoclonus Syndrome Treatment - Opsoclonus Myoclonus Syndrome Resources - Opsoclonus Myoclonus Syndrome Not supplied. [checkorphan.org]

  From Wikipedia, the free encyclopedia Opsoclonus myoclonus syndrome Other names Dancing eye syndrome[1] Specialty Neurology Opsoclonus myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia (OMA), is a rare neurological disorder of unknown [en.wikipedia.org]

• Cerebellar Ataxia

  Neuroimaging is abnormal and the prognosis is less favorable than in acute cerebellar ataxia. [scienceopen.com]

  Abstract Background : Opsoclonus myoclonus syndrome is a rare condition characterized by the amalgamation of multidirectional saccades of eye movements, involuntary myoclonus predominantly affecting trunk and limbs and cerebellar ataxia. [casereports.in]

• Nystagmus

  Due to the persistent ataxia and nystagmus, a history of heavy smoking, and weight loss, hematology and oncology was consulted and a paraneoplastic work-up was done. [clinmedjournals.org]

  Nystagmus is defined by the presence of a slow phase during the eye movement, which may or may not be followed by a fast refixating movement (jerk nystagmus). When only consisting of slow to-and-fro movements, it is known as pendular nystagmus. [aao.org]

  Ocular nystagmus - myoclonus syndrome (OMS - Opsoclonus - myoclonus syndrome) or nystagmus - myoclonus - ataxia is a syndrome that includes the following manifestations: nystagmus, focal or diffuse myoclonus, unsteady gait or may be accompanied by other [vinmec.com]

  The mildest cases of acute cerebellar ataxia represent a benign condition that is characterized by acute truncal and gait ataxia, variably with appendicular ataxia, nystagmus, dysarthria, and hypotonia. [ncbi.nlm.nih.gov]

  Differential diagnosis Differential diagnosis includes acute inflammatory cerebellar ataxia that is differentiated from OMS by the type of eye movement (nystagmus), the absence of irritability, and the usually rapid recovery without treatment. [orpha.net]

• Ocular Flutter

  Both ocular flutter and opsoclonus may occur with myoclonus and ataxia. The pathophysiology of ocular flutter is also poorly understood (1). It may be difficult to distinguish these saccadic intrusions from nystagmus. [eyerounds.org]

  Saccadic movements are termed opsoclonus when multidirectional and ocular flutter when in horizontal plane. [n.neurology.org]

  A 67-year-old woman presented with a complex partial seizure and evolving ocular flutter, opsoclonus, myoclonus and 'cerebellar' signs, all of which improved spontaneously within 6 weeks. [ncbi.nlm.nih.gov]

  It should be noted that ocular flutter and opsoclonus fall along a spectrum, and it is possible for ocular flutter to progress to opsoclonus. Thus, ocular flutter in the appropriate clinical context should also raise suspicion for OMS. [aao.org]

  Read the full text or download the PDF: Log in using your username and password Read the full text or download the PDF: Log in using your username and password Other content recommended for you Sequential fluctuating paraneoplastic ocular flutter–opsoclonus–myoclonus [pn.bmj.com]

• Truncal Ataxia

  ataxia (2.5 years ) 7 50/M Monophasic Corticosteroids > 3 months Asymptomatic (4 years) 8 53/M Monophasic Azathioprine > 3 months Mild truncal ataxia(10 years) 9 76/F Relapsing IVIG at relapse Remission (see text) Mild truncal ataxia (4 years) 10 80/ [academic.oup.com]

  Opsoclonus, myoclonus, and truncal ataxia were noted. Two weeks after treatment with intravenous immunoglobulin and corticosteroids, her opsoclonus, myoclonus, and truncal ataxia disappeared. [ncbi.nlm.nih.gov]

  Neurological examination revealed coarse eye movements with severe truncal ataxia and limb myoclonus. [neurologyindia.com]

The main tool for establishing this diagnosis is a clinical examination, with careful observation of symptoms described above. If tumors are suspected, computer tomography scans of the neck, chest, abdomen and pelvis, as well as iodine-123-meta-iodobenzylguanidine scintigraphic scans should be performed in children, whereas adults benefit from positron emission tomography.

Laboratory workup includes oligoclonal bands, lymphocyte subset analysis showing a high number of CSF B cells, studies of cytokines and chemokines and autoantibody 9Hu antineuronal nuclear antibodies. Although autoantibody testing is not routinely recommended, tests for autoantibodies directed against cerebellar Purkinje cells as well as peripheral nerve axons can be performed [7] [8], in addition to the routine complete blood cell count. Other autoantibodies that have been described in this context are: neuronal nuclear antigens [9] [10], anti-neuronal calcium channels [11] [12], N-methyl-D-aspartate receptor [13] [14] and ANNA-2 autoantibodies.

The presence of an infectious etiology should also be established (Epstein-Barr virus, cytomegalovirus, rickettsial infection, mycoplasma, Whipple disease, herpes simplex virus, varicella zoster virus and human immunodeficiency virus). Furthermore, the clinician should keep in mind that cases have also been described in association with hyperosmolar nonketotic diabetic coma and cocaine ingestion. If tests fail to demonstrate a neoplastic or infectious etiology, an autoimmune reaction against central nervous system tissues should be suspected.

If a spinal puncture is performed, one can expect to find higher amounts of B cells in the cerebrospinal fluid of opsoclonus-myoclonus syndrome patients, probably in a neuroinflammatory context [15].

Urinary workup should test the levels of vanillyl mandelic and homovanillic acid, which have been reported to be high in neuroblastoma patients.

• Human Herpesvirus 6

  Neurology 68:1636 Article CAS PubMed Google Scholar Crawford JR, Kadom N, Santi MR, Mariani B, Lavenstein BL (2007) Human herpesvirus 6 rhombencephalitis in immunocompetent children. [link.springer.com]

  Human herpesvirus 6 rhombencephalitis in immunocompetent children. J Child Neurol. 2007 Nov;22(11):1260-8. 27. Zaganas I, Prinianakis G, Xirouchaki N, Mavridis M. Opsoclonus-myoclonus syndrome associated with cytomegalovirus encephalitis. [neuroophthalmology.ca]

Treatment There is no known definitive cure for OMS. However, several drugs have proven to be effective in its treatment. [bionity.com]

Please also consider donating plasma to the blood bank (each dose of IVIG treatment is made from the plasma of approximately 1000 donors). [youcaring.com]

treatment modality in OMS. [mdpi.com]

Management and treatment Treatment usually includes resection of the neuroblastoma if present; occasionally, higher grade neuroblastoma may require chemotherapy. Treatment also includes immunomodulation. [orpha.net]

The CD4 + T cell/CD8 + T cell ratio, which is characteristically low in OMS, was 1.3 before rituximab treatment and 0.7 after rituximab treatment, compared with 3.8 ± 0.7 in control samples. [doi.org]

Diagnosis - Opsoclonus Myoclonus Syndrome Prognosis - Opsoclonus Myoclonus Syndrome Treatment - Opsoclonus Myoclonus Syndrome Resources - Opsoclonus Myoclonus Syndrome Not supplied. [checkorphan.org]

Prognosis Currently there are no clinically established laboratory investigations available to predict prognosis or therapeutic response. [bionity.com]

This finding is consistent with recent studies that suggest multimodal immunotherapy regimens may be improving the prognosis in this challenging disease. [ncbi.nlm.nih.gov]

Neuroblastoma in a child Most adults with opsoclonus myoclonus have neoplastic, infectious, metabolic, or idiopathic etiologies. Small Cell Lung Carcinoma in an adult [pathology-india.com]

Opsoclonus-myoclonus syndrome (OMS) may have a toxin induced, parainfectious, or paraneoplastic etiology. [ncbi.nlm.nih.gov]

Etiology/Epidemiology OMS is exceedingly rare, with an estimated incidence of 1 per 5 million people per year (2). The etiologies of OMS may be divided into three main categories: paraneoplastic, para-infectious, and idiopathic. [eyerounds.org]

Its etiology may be paraneoplasic (neuroblastoma), non-paraneoplasic (infectious processes) or idiopathic. [pesquisa.bvsalud.org]

Etiology OMS may have a paraneoplastic, parainfectious or idiopathic origin. In the majority of pediatric paraneoplastic cases, a neuroblastoma is found. [orpha.net]

The aim of our study was to investigate the epidemiology, clinical features, etiological aspects and outcome of OMS in Tunisian children. [ncbi.nlm.nih.gov]

Conclusion | Epidemiological Evidence | Proposed Biological Mechanism | Archives | References Conclusion Opsoclonus myoclonus syndrome (OMS) is a very rare neurological condition that generally begins at one to two years of age and is characterized by [vaccinesafety.edu]

The descriptions of the clinical conditions each systematically cover, as appropriate, epidemiology, prevalence, diagnostic criteria, clinical features (including course and prognosis), pathophysiology, genetics, investigations, differential diagnosis [mackeith.co.uk]

The antibodies associated with some cases of OMS are thought to play a role in the pathophysiology of the syndrome although the exact immunologic mechanism remains unknown. [ncbi.nlm.nih.gov]

The aim of this article is to review opsoclonus, with particular emphasis on its immunopathogenesis and pathophysiology. [scienceopen.com]

Pathophysiology The pathophysiology of OMS remains largely unknown but is thought to be related to dysfunction in the generation of saccades. There are two leading hypotheses. The first points to cerebellar dysfunction. [eyerounds.org]

Symptoms - Opsoclonus Myoclonus Syndrome Causes - Opsoclonus Myoclonus Syndrome Prevention - Opsoclonus Myoclonus Syndrome Not supplied. [checkorphan.org]

The movement disorder prevented him from walking, writing, and eating and did not improve with corticosteroid therapy. The patient's cerebellar function. .. Mathias Nitschke, M.D. Fred Hochberg, M.D. [nejm.org]

The second hypothesis implicates damage to omnipause cells, which normally inhibit saccade-generating burst neurons in the parapontine reticular formation and prevent unwanted saccades. [eyerounds.org]

Conventional immunotherapies often do not prevent relapse or permanent sequelae. To test the cellular immune hypothesis of OMS in a cross-sectional study and determine if CSF lymphocyte subset analysis provides biomarkers of disease activity. [ncbi.nlm.nih.gov]

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