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Opsoclonus-Myoclonus Syndrome

OMS

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.


Presentation

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.

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.[ncbi.nlm.nih.gov]
Turkish
  • Gure, Frequency of SOX Group B (SOX1, 2, 3) and ZIC2 antibodies in Turkish patients with small cell lung carcinoma and their correlation with clinical parameters, Cancer, 103, 12, (2575-2583), (2005).[doi.org]
Low Fever
  • 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]
Sputum
  • Sorological tests and sputum Mycoplasma real-time PCR were positive on seventh day in-hospital. Patient was treated with Azithromycin and IV Immunoglobulin for five days.[ncbi.nlm.nih.gov]
Bilious Vomiting
  • The fever was associated with nausea and a few episodes of non-projectile, non-bilious vomiting. On the fifth day of fever she developed tremulousness of the whole body and a staggering gait.[doi.org]
Hepatosplenomegaly
  • The rest of the neurological and systemic examination was normal, except for mild hepatosplenomegaly.[doi.org]
Otalgia
  • A 50-year-old British Caucasian woman presented with left-sided otalgia and subjective hearing loss. Over the coming weeks she developed subacute confusion and dizziness, leading to recurrent falls.[ncbi.nlm.nih.gov]
Nystagmus
  • 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]
  • 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]
Myoclonic Jerking
  • Clinical features of OMS include opsoclonus with myoclonic jerks and cerebellar ataxia.[ncbi.nlm.nih.gov]
  • BACKGROUND: Kinsbourne syndrome or opsoclonus myoclonus syndrome (OMS) is characterized by rapid, involuntary, irregular conjugate eye movements (opsoclonus), myoclonic jerking of the limbs and trunk, ataxia, and behavioral disturbances.[ncbi.nlm.nih.gov]
  • Immediately after she complained of left-sided paraesthesia and later became disoriented, with incoherent speech, inability to obey commands, opsoclonus of the eyes and myoclonic jerks.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
Mental Deterioration
  • In order to prevent further clinical and mental deterioration, 6 pulses of cyclophosphamide in combination with dexamethasone pulses every 4 weeks were administered. Both children showed significant improvement of OMS symptoms.[ncbi.nlm.nih.gov]
Mononeuropathy
  • A 37-year-old woman with previous inflammatory cranial mononeuropathies was admitted for elective dilatation and curettage (D&C).[ncbi.nlm.nih.gov]
Apathy
  • Patients also present with behavior disturbances such as rage attacks, irritability or apathy, and sleep pattern changes.[symptoma.com]

Workup

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.

Lymphocytic Infiltrate
  • Diffuse and extensive lymphocytic infiltration with lymphoid follicles is a characteristic histologic feature of neuroblastic tumors with opsoclonus-myoclonus.[ncbi.nlm.nih.gov]
  • The detection of autoantibodies and lymphocytic infiltration in NB patients led to advance an autoimmune hypothesis for the pathogenesis of OMS-related NB.[ncbi.nlm.nih.gov]
  • Conclusions Diffuse and extensive lymphocytic infiltration with lymphoid follicles is a characteristic histologic feature of neuroblastic tumors with opsoclonus–myoclonus.[doi.org]

Treatment

  • 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]
  • This time, we only administered an additional single dose of RTX treatment (375 mg/m2), allowing remission of OMS symptoms. During 2 years after the additional RTX treatment, OMS symptoms did not appear, even when prednisolone was reduced.[ncbi.nlm.nih.gov]
  • Due to the possible immune-mediated mechanisms, treatment with steroids, ACTH, plasmapheresis and immunoglobulins can be successful. However, some children become steroid dependent and symptoms may reoccur after treatment has been finished.[ncbi.nlm.nih.gov]
  • Without appropriate treatment for SCLC, all reported patients with SCLC and OMS have died of complications of OMS within 3 months of diagnosis.[ncbi.nlm.nih.gov]
  • Demographic data, neurological and oncological status, histopathology, treatments, prognosis, and diagnosis and treatment timing were retrospectively reviewed from the records.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • We reviewed the neurological and oncological outcomes of NT with OMS, and discussed whether the treatment of NT would contribute to improving the neurological prognosis.[ncbi.nlm.nih.gov]
  • The prognosis for the opsoclonus myoclonus syndrome in children with Epstein-Barr virus infection and without evidence for a neuroblastoma appears to be better than in those patients where a cause cannot be identified.[ncbi.nlm.nih.gov]
  • Monophasic disease course and full recovery correspond to the favorable prognosis known from parainfectious cases in young adults. This should affect therapeutic consideration.[ncbi.nlm.nih.gov]
  • Prompt diagnosis of this disorder is important because after tumor removal and immunomodulatory therapies it has a relatively good prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Opsoclonus-myoclonus syndrome (OMS) may have a toxin induced, parainfectious, or paraneoplastic etiology.[ncbi.nlm.nih.gov]
  • Since autoantibodies have been detected in some patients with OMS, an autoimmune etiology is suspected.[ncbi.nlm.nih.gov]
  • If tests fail to demonstrate a neoplastic or infectious etiology, an autoimmune reaction against central nervous system tissues should be suspected.[symptoma.com]
  • Etiology OMS may have a paraneoplastic, parainfectious or idiopathic origin. In the majority of pediatric paraneoplastic cases, a neuroblastoma is found.[orpha.net]
  • There was no evidence for any other etiology responsible for her opsoclonus-myoclonus syndrome. Her opsoclonus-myoclonus syndrome appeared associated with West Nile encephalitis and had an unfavorable evolution despite treatment.[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • Worldwide, data on its epidemiology, clinical features, and outcome are scarce. The aim of the study was to determine the clinical profile and outcome of children with this disorder.[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]
  • The aim of our study was to investigate the epidemiological characteristics of OMS in Japan and to clarify the association between therapy and prognosis.[ncbi.nlm.nih.gov]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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 pathophysiology is thought to be immunological on the basis of the paraneoplasticity and the symptomatic (though often incomplete) response to immunomodulatory therapies; a number of autoantibodies have been identified to a variety of antigens and[ncbi.nlm.nih.gov]
  • This finding highlights the usefulness of functional/metabolic brain imaging to study the pathophysiology of this type of disorder.[ncbi.nlm.nih.gov]
  • We suggest that in HIV-associated OMS the pathophysiology may be the consequence of a dysregulated immune system in which a reduced CD4/CD8 ratio, in addition to a critical level of functional CD4 cells for efficient CD8 cytotoxicity, results in dysfunction[ncbi.nlm.nih.gov]
  • An autoimmune pathophysiology is suspected because childhood OMS patients have functionally active autoantibodies, proinflammatory changes in the cytokine network and immunotherapy responses.[ncbi.nlm.nih.gov]

Prevention

  • 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]
  • In order to prevent further clinical and mental deterioration, 6 pulses of cyclophosphamide in combination with dexamethasone pulses every 4 weeks were administered. Both children showed significant improvement of OMS symptoms.[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]
  • Conventional immunotherapies often do not prevent relapse or permanent sequelae.[doi.org]
  • […] diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PS parasitology PA pathology PP physiopathology PC prevention[decs.bvs.br]

References

Article

  1. Klaas JP, Ahlskog JE, Pittock SJ, et al. Adult-onset opsoclonus-myoclonus syndrome. Arch Neurol. 2012; 69:1598-1607.
  2. Matthay KK, Blaes F, Hero B, et al. Opsoclonus myoclonus syndrome in neuroblastoma: a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. Cancer Letters. 2005; 228:275-282.
  3. Jung KY, Youn J, Chung CS. Opsoclonus-myoclonus syndrome in an adult with malignant melanoma. J Neurol. 2006;253(7):942-943.
  4. Moretti R, Torre P, Antonello RM, Nasuelli D, Cazzato G. Opsoclonus-myoclonus syndrome: gabapentin as a new therapeutic proposal. Eur J Neurol. 2000;7(4):455-456.
  5. De Luca S, Terrone C, Crivellaro S, et al. Opsoclonus-myoclonus syndrome as a paraneoplastic manifestation of renal cell carcinoma: a case report and review of the literature. Urol Int. 2002;68(3):206-208.
  6. Kumar A, Lajara-Nanson WA, Neilson RW Jr. Paraneoplastic opsoclonus-myoclonus syndrome: initial presentation of non-Hodgkin's lymphoma. J Neurooncol. 2005;73(1):43-45.
  7. Connolly AM, Pestronk A, Mehta S, et al. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: targets in neural tissues. J Pediatr. 1997;130:878-884.
  8. Antunes NL, Khakoo Y, Matthay KK, et al. Antineuronal antibodies in patients with neuroblastoma and paraneoplastic opsoclonus-myoclonus. J Pediatr Hematol Oncol. 2000; 22:315-320.
  9. 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):437-443.
  10. Hersh B, Dalmau J, Dangond F, et al. Paraneoplastic opsoclonus-myoclonus associated with anti-Hu antibody. Neurology. 1994;44(9):1754-1755.
  11. Josephson CB, Grant I, Benstead T. Opsoclonus-myoclonus with multiple paraneoplastic syndromes and VGCC antibodies. Can J Neurol Sci. 2009;36(4):512-514.
  12. Simister RJ, Ng K, Lang B, et al. Sequential fluctuating paraneoplastic ocular flutter-opsoclonus-myoclonus syndrome and Lambert-Eaton myasthenic syndrome in small-cell lung cancer. J Neurol Neurosurg Psychiatry. 2011;82(3):344-346.
  13. Smith JH, Dhamija R, Moseley BD, et al. N-methyl-D-aspartate receptor autoimmune encephalitis presenting with opsoclonus-myoclonus: treatment response to plasmapheresis. Arch Neurol. 2011;68(8):1069-1072.
  14. Kurian M, Lalive PH, Dalmau JO, et al. Opsoclonus-myoclonus syndrome in anti- N-methyl-D-aspartate receptor encephalitis. Arch Neurol. 2010;67(1):118-121.
  15. Pranzatelli MR, Travelstead AL, Tate ED, et al. CSF B-cell expansion in opsoclonus-myoclonus syndrome: a biomarker of disease activity. Mov Disord. 2004;19:770-777.

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Last updated: 2019-07-11 20:16