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Opsoclonus


Presentation

  • 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]
  • The duration of symptoms at the time of presentation was in the range of 6–10 months.[pediatricneurosciences.com]
  • 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]
  • 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]
Enuresis
  • Children were difficult to toilet train, and some older children had persistent enuresis (3/17) or encopresis (1/3). School age patients were in special education classes, and attentional problems and hyperactivity were reported in school.[neuro.psychiatryonline.org]
Splenomegaly
  • Remaining neurological and systemic examinations were normal, except there was mild splenomegaly. He had received a course of chloroquine (1500 mg of base given over 48 hours) prior to admission. All routine blood and urine parameters were normal.[ijo.in]
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]
Dysphoric Mood
  • Persistent irritability, dysphoric mood and poor affective regulation (10/17) were noted at psychiatric evaluation. Night terrors continued in some patients (4/17).[neuro.psychiatryonline.org]
Head Banging
  • One child (case 1) also received intravenous immunoglobulin (IVIG) and oral clonazepam and risperidone in view of abnormal behavior (excessive irritability, biting, and head banging).[pediatricneurosciences.com]
Tantrums
  • While Keira didn’t exhibit that symptom, she had the other hallmarks of an attack on the body’s motor system: an inability to walk, trembling hands and the full-volume tantrums that are inconsolable.[globalgenes.org]
  • Disruptive behavior problems with severe tantrums, restiveness, and aggressive and/or self-injurious behaviors were noted in one-half of the patients (8/17).[neuro.psychiatryonline.org]
Motor Symptoms
  • Parents noted that subsequent steroid therapy seemed to worsen irritability, lability and aggression, although motor symptoms improved.[neuro.psychiatryonline.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]
Incontinence
  • CASE REPORT A 49-year-old woman with a past medical history of depressive disorder and bilateral breast reduction mammoplasty was admitted to anal sphincteroplasty for the treatment of fecal incontinence as a labor complication.[academic.oup.com]

Workup

  • 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]

Treatment

  • 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]
  • 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]
  • 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]
  • 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]

Prognosis

  • 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]

Etiology

  • 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]

Epidemiology

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

Pathophysiology

  • 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]
  • The exact pathophysiology of OMS is unknown; however, there are two proposed mechanisms.[aao.org]

Prevention

  • 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]
  • The aims of treatment are firstly to treat the neuroblastoma tumour if present and secondly to attacks to the cerebellum and prevent any lasting damage.[gosh.nhs.uk]

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