Symptoma

Opsoclonus

The clinical features described above may not always be present upon patient presentation, posing a diagnostic challenge. Because of this variability in presentation, a set of diagnostic criteria has been proposed by international collaborators. [aao.org]

The duration of symptoms at the time of presentation was in the range of 6–10 months. [pediatricneurosciences.com]

We present a case of scrub typhus with a rare ophthalmic manifestation.Our patient presented with fever and opsoclonus, was diagnosed to have scrub typhus and completely improved upon treatment. [jpgmonline.com]

Clinical description OMS typically presents between 1 and 3 years of age, although it can occur earlier or later in childhood. [orpha.net]

Here we present the case of a girl of 15 months with OMS associated with retroperitoneal ganglioneuroblastoma that improved after tumor resection and treatment with corticosteroids and immunoglobulin. [scielo.org.co]

  • Crying

    Behavioral and sleep disturbances, including extreme irritability, inconsolable crying, reduced and fragmented sleep (insomnia) and rage attacks are common. [rarediseases.org]

  • Difficulty Walking

    The impact on the cerebellum causes the patient to develop difficulty walking, chaotic eye movements, and a loss of developmental skills. [chop.edu]

  • Dysphagia

    […] saccades), truncal instability, cerebellar ataxia, and diffuse myoclonus. 1–3 In addition to these classic manifestations, the condition may also be associated with irritability, alterations in the sleep-wake cycle, headache, language or visual disorders, dysphagia [elsevier.es]

  • Hiccup

    They may also be physiologic or benign (eg, hiccups). Opsoclonus-myoclonus syndrome Opsoclonus-myoclonus syndrome (OMS) is a rare condition of unknown etiology that features opsoclonus, myoclonic jerks, behavioral disturbances, and ataxia. [aao.org]

  • Ear Fullness

    Labyrinthitis is a rare complication of acute otitis media that can produce nystagmus and vertigo with secondary ataxia. 10 Patients often have fever, ear fullness, and pain, as well as mastoiditis. Opsoclonus is not a feature of labyrinthitis. [aao.org]

  • Anger

    Angers, France, 49933 Contact: PELLIER Isabelle, DR Principal Investigator: PELLIER Isabelle, DR Hopital Jean Minjoz Besancon, France, 25030 Contact: KLEIN Stéphane, DR Principal Investigator: KLEIN Stéphane, DR Chr Pellegrin Bordeaux, France, 33076 [clinicaltrials.gov]

    Feelings of anger, anxiety, fear and sadness, for example, are common and understanding and managing these may be easier with the support of the Clinical Psychologist. [gosh.nhs.uk]

  • Apathy

    Attempts to move or maintain certain postures are often disrupted by myoclonic jerking movements that may include the head, trunk, or extremities • Behavioral and sleep disturbances as well as profound irritability, sometimes being inconsolable • Sometimes apathy [slideshare.net]

    In contrast, there have also been descriptions of apathy or social withdrawal. Opsoclonus is generally observed within days after the onset of ataxia, but has been reported to occur weeks to months later in some cases. [aao.org]

  • Clumsiness

    He has had a 1-2 week history of increased “clumsiness” and has fallen several times while walking. They also comment that he has been much more irritable lately and has not slept well. He has been previously healthy. [aao.org]

  • Loss of Speech

    Difficulty articulating speech (dysarthria), sometimes with complete loss of speech and language may occur. Additional symptoms such as decreased muscle tone (hypotonia) and vomiting are common. [rarediseases.org]

  • Guillain-Barré Syndrome

    Patients were also misdiagnosed with labyrinthitis and Guillain-Barré syndrome (GBS). In cases where opsoclonus is either absent or delayed in onset, acute cerebellar ataxia or acute cerebellitis of childhood can be easily confused with OMS. [aao.org]

For etiological clarification, a full workup was conducted with blood tests, including tumor markers and serology for human immunodeficiency virus, cytomegalovirus and Epstein Barr virus; brain magnetic resonance imaging (MRI), CT scan of the chest, abdomen [academic.oup.com]

In older adults, a full-scale workup for neoplasm is generally indicated and is productive in persons with opsoclonus. [dizziness-and-balance.com]

  • Gliosis

    Histopathologic examination of patients with OMS has demonstrated gliosis and inflammation in the cerebellar vermis, supporting this theory. [aao.org]

After 2 years of treatment, the patient is in complete remission and has no sequelae. Multiple treatment protocols for OMAS have been developed. [elsevier.es]

Treatment Treatment for opsoclonus myoclonus may include corticosteroids or ACTH (adrenocorticotropic hormone). In cases where there is a tumor present, treatment such as chemotherapy, surgery, or radiation may be required. [childneurologyfoundation.org]

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]

There is controversy about how treatment alters outcome. [pediatrics.aappublications.org]

This protocol will investigate an escalating treatment schedule starting with a corticosteroid standard treatment with dexamethasone pulses (first step), which is followed, if response has been inadequate after 3 months of treatment, by the addition of [centerwatch.com]

Prognosis The prognosis for opsoclonus myoclonus varies depending on the symptoms and the presence and treatment of tumors. With treatment of the underlying cause of the disorder, there may be an improvement of symptoms. [childneurologyfoundation.org]

PROGNOSIS • The survival prognosis of children with OMS secondary to neuroblastoma is very favorable better than neuroblastoma without OMS • A monophasic course and that those patients generally had a more favorable neurologic prognosis than patients [slideshare.net]

While the OMS is not a common pediatric pathology, its association with neoplastic disease is very strong and ignore its existence and clinical presentation may lead to delays or underdiagnosis of the tumor with negative impact on the prognosis of the [scielo.org.co]

There are no known criteria to predict prognosis. Corticosteroidor corticotropin are recommended treatment regimes. Conclusions.The boy with OMAS is described. The diagnosis was made in retrospect. [worldcat.org]

Trazodone has been reported to be beneficial and well tolerated for treatment of associated sleep disturbances or rage attacks. 29 Prognosis Overall, the survival prognosis of children with OMS secondary to neuroblastoma is very favorable. [aao.org]

The etiology was paraneoplastic in 13 (35.1%) cases, neuroblastoma being the most common tumor; a post-infectious etiology was seen in 5 cases; other etiologies were cerebral malaria, dengue, human immunodeficiency virus infection, scrub typhus and poisoning [neurologyindia.com]

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

After infectious and post-infectious etiologies were excluded the patient was screened for autoimmune and drug-related etiologies, without success. [karger.com]

There are reports of certain auto-antibodies associated with OMS, supporting the suspected etiology of an autoimmune reaction against central nervous system tissues. [aao.org]

Immunotherapy should be initiated along with a complete work up for underlying malignancy, infectious, systemic diseases and toxic etiology. [file.scirp.org]

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]

OUTLINE: • Introduction • Pathophysiology • Epidemiology • Clinical presentation • Diagnostic criteria • Differential diagnosis • Etiology / risk factors • Clinical work-up / diagnostic • Management • Prognosis • Summary 3. [slideshare.net]

Epidemiologic data show that opsoclonus myoclonus ataxia syndrome affects about 2–3% of patients with neuroblastoma, and neuroblastoma are found in about 40% of patients with opsoclonus myoclonus ataxia syndrome. [mdedge.com]

Sri Lanka: Epidemiology Unit; 2008. 2. Wang H, Liang G. Epidemiology of Japanese encephalitis: Past, present, and future prospects. Ther Clin Risk Manag. 2015;11:435–48. 3. Misra UK, Kalita J. [jmedicalcasereports.biomedcentral.com]

Epidemiology syndrome due to its rarity poorly understood. In most cases, the OMS is paraneoplastic process in which a 43-80% can be revealed neuroblastoma (NB) [1,2]. Neuroblastoma associated with the OMS, has a number of... [posterng.netkey.at]

OUTLINE: • Introduction • Pathophysiology • Epidemiology • Clinical presentation • Diagnostic criteria • Differential diagnosis • Etiology / risk factors • Clinical work-up / diagnostic • Management • Prognosis • Summary 3. [slideshare.net]

[…] treatment approach constitutes surgical resection of the tumour followed by immunomodulatory therapy with ACTH and IVIg. 3 Due to the aggressiveness of the syndrome, however, treatment aims to reduce the formation of antibodies potentially involved in the pathophysiology [elsevier.es]

While the pathophysiology is unknown, OMS is felt to be related to autoimmunity. As a result of cerebellar Purkinje cell dysfunction, oculomotor neurons of the caudal fastigial nucleus of the cerebellum become progressively disinhibited. [radiopaedia.org]

[…] in the chronic stage as the symptoms resolved. [29] Another magnetic resonance imaging-based study noted a reduced connectivity between the cerebellum and motor cortex, but an increased connectivity with occipito-parietal regions. [30] An autoimmune pathophysiology [neurologyindia.com]

Treatment with immunosuppressants is commonly used in patients with Kinsbourne syndrome regardless of etiology, and aims to reduce the formation of antibodies possibly involved in the pathophysiology 1,3. [scielo.br]

These studies are aimed at increasing knowledge about these disorders and finding ways to prevent, treat, and cure them. [childneurologyfoundation.org]

Opsoclonus-ataxia caused by neuroblastoma causes substantial developmental sequelae that are not adequately prevented by current treatment. [pediatrics.aappublications.org]

Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [edhub.ama-assn.org]

Treatment entails a nonspecific approach to immune therapy, with the aim of reducing neuroinflammation, inducing complete remission, and preventing relapses. Relapses occur in approximately half of patients, Dr. Dale said. [mdedge.com]

These were not included in the calculation of mean to prevent an outlier effect. In the other 11 patients, the mean CSF WBC count was 9 2.7 cells/cumm with lymphocytic pleocytosis in all samples. [annalsofian.org]