Opsoclonus-myoclonus syndrome (also known as dancing eyes- dancing feet syndrome) is a rare disease of inflammatory, parainfectious, paraneoplastic or idiopathic nature consisting of neurologic and behavioral disorders.
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 , head tilt, excessive drooling, malaise, incoordination, cranial nerve palsies, seizures and sleep disturbances . 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.
Opsoclonus-myoclonus syndrome is usually associated with neuroblastoma in children  and small-cell lung cancer or breast adenocarcinoma in adults. Other conditions that should be excluded are melanoma , gynecologic malignancies , urologic cancers , hematologic  and gastrointestinal neoplasms.
Entire Body System
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]
Review of Systems Positive for recent upper respiratory illness symptoms, fatigue, night sweats, nausea, and vomiting. Negative for acute decrease in vision. [webeye.ophth.uiowa.edu]
It is genetically engineered from murine cells, the proteins of which can cause hypersensitivity. 2 We observed none of the possible infusion-related symptoms, such as fever, chills, nausea, hives, fatigue, headache, or itching, probably because of our [doi.org]
Other infusion-related symptoms include nausea, hives, fatigue, headache, and itching. Symptoms resolve with slowing of the infusion and treatment with acetaminophen (Tylenol) and IV saline and Benadryl. [omsusa.org]
Other symptoms that developed included weight loss ( 4.5 kg), 10 patients; mood changes, 9 (depression, 5; irritability/emotional lability, 4); dysphagia, 3 (resulting from myoclonus or nausea/vomiting); fatigue/sleep disturbance, 3; headache, 1; and [jamanetwork.com]
Varicella-Zoster Virus Infection
We report a case of OMS in a patient with varicella zoster virus infection. IgM antibody for varicella-zoster virus was detected in the serum and the cerebrospinal fluid. [doi.org]
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). [symptoma.com]
Infections for which testing was performed in 18 patients (serum, 13; CSF, 13) included Epstein-Barr virus, 13 patients; cytomegalovirus, 11; rickettsial infection, 8; mycoplasma, 5, Whipple disease, 4; herpes simplex virus, 4; varicella zoster virus, [jamanetwork.com]
We report a case of a 68 years-old woman, with previous history of diabetes mellitus and systemic hypertension that was referred for evaluation due to headache and low fever for three days. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
We also did not encounter rare but more serious hypersensitivity reactions such as hypotension, bronchospasm, or angioedema. [doi.org]
Rituxan can be associated with more serious hypersensitivity reactions such as low blood pressure (hypotension), breathing difficulties (bronchospasm), and sensation of tongue or throat swelling (angioedema). [omsusa.org]
Neuroimaging is abnormal and the prognosis is less favorable than in acute cerebellar ataxia. [ncbi.nlm.nih.gov]
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]
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]
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]
Opsoclonus refers to dancing eyes which in medical terms is known as Nystagmus. Myoclonus is involuntary contraction and twitching of muscles. The other names for this condition are Kinsbourne syndrome or ‘dancing eye-dancing feet’ syndrome. [tandurust.com]
A 29-month-old girl with worsening ataxia, nystagmus, and subsequent opsoclonus and myoclonus. New Engl J Med 333:579–586 Google Scholar 18. Hattner RS, Pounds TR, Matthay KK (1994) Normal cerebellar MIBG localization. [doi.org]
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]
Clinical features of OMS include opsoclonus with myoclonic jerks and cerebellar ataxia. [ncbi.nlm.nih.gov]
Neurological examination revealed abnormal chaotic multidirectional movement of eyes suggestive of opsoclonus with evidence of myoclonic jerks involving all four limbs, face and eyelids [Videos 1 and 2]. [ruralneuropractice.com]
It is characterized by opsoclonus (rapid, multi-directional, conjugate eye movements), myoclonic jerks, ataxia, irritability and sleep disturbances. The clinical course may be monophasic or chronic relapsing. [orpha.net]
The patient's condition worsened rapidly to an akinetic mutism, followed by a period of agitation, delirium, and hallucinations. [ncbi.nlm.nih.gov]
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  , in addition to the routine complete blood cell count. Other autoantibodies that have been described in this context are: neuronal nuclear antigens  , anti-neuronal calcium channels  , N-methyl-D-aspartate receptor   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 .
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]
Complete remissions were obtained in 35.3%, 23.1%, 33.3%, 66.7%, and 100% of these treatments, respectively. [ncbi.nlm.nih.gov]
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]
Opsoclonus-myoclonus syndrome (OMS) may have a toxin induced, parainfectious, or paraneoplastic etiology. [ncbi.nlm.nih.gov]
Etiology OMS may have a paraneoplastic, parainfectious or idiopathic origin. In the majority of pediatric paraneoplastic cases, a neuroblastoma is found. [orpha.net]
If tests fail to demonstrate a neoplastic or infectious etiology, an autoimmune reaction against central nervous system tissues should be suspected. [symptoma.com]
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 uncontrolled [vaccinesafety.edu]
Summary Epidemiology The annual incidence is estimated at around 1/5,000,000. Clinical description OMS typically presents between 1 and 3 years of age, although it can occur earlier or later in childhood. [orpha.net]
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]
Early effective treatments within 30 weeks after the onset may be required to prevent the serious neurological outcome. Copyright 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved. [ncbi.nlm.nih.gov]
Symptoms - Opsoclonus Myoclonus Syndrome Causes - Opsoclonus Myoclonus Syndrome Prevention - Opsoclonus Myoclonus Syndrome Not supplied. [checkorphan.org]
Conventional immunotherapies often do not prevent relapse or permanent sequelae. [doi.org]
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