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]

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]

• Movement Disorder

  Our video documentation will aid to recognize this rare movement disorder and to initiate early treatment. Background The spectrum of movement disorders in childhood is diverse. Diagnosis remains often descriptive. [springerplus.springeropen.com]

  Research The NINDS supports and conducts research on movement disorders such as opsoclonus myoclonus. These studies are aimed at increasing knowledge about these disorders and finding ways to prevent, treat, and cure them. [childneurologyfoundation.org]

  Antibodies have been shown to bind to cerebellar neurons and cause a transient disorder, resulting in a temporary movement disorder. [jpgmonline.com]

  Variantes de point d'accès Ocular Motility Disorder [Nom commun] Eye Movement Disorders [Nom commun] Eye Movement Disorder [Nom commun] Eye Motility Disorders [Nom commun] Eye Motility Disorder [Nom commun] Troubles de l'appareil moteur de l'oeil [Nom [idref.fr]

  Learn more about CME/MOC Related Articles Opsoclonus-Myoclonus Syndrome in Anti–N-Methyl-D-Aspartate Receptor Encephalitis See More About Movement Disorders Neurology Ophthalmology Inflammatory CNS Disorders X Button To Top Close Sign in to customize [edhub.ama-assn.org]

• Fever

  Case presentation Here we present the case of a 31-year-old Sri Lankan woman who presented with fever, altered level of consciousness, opsoclonus, and facial myoclonus. [jmedicalcasereports.biomedcentral.com]

  Opsoclonus was first noted after a median interval of 11 (7–18) days from fever onset. [annalsofian.org]

  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]

  Those with severe symptoms have the highest risk for permanent neurological problems. [3] Relapses of neurological symptoms may occur with future illness, fever, stress, medication, anesthesia, discontinuation of immunotherapy, or after immunizations. [rarediseases.info.nih.gov]

  Case report A 36 year old man complained of high grade fever of 7 days duration which persisted till 3 days prior to admission, when he developed complaint of blurring and shaking of vision. [ijo.in]

• Inflammation

  After two surgeries, the woman died unexpectedly and the autopsy showed inflammation of the cranial pia mater as the cause of the cyst [1]. [ncbi.nlm.nih.gov]

  Acute cerebellar ataxia represents cerebellar inflammation that typically occurs following infection or immunization. [aao.org]

  Another fact confirming a predominant cerebellar inflammation is the brain MRI on 5-year follow-up with mild signs of cerebellar atrophy—a finding previously reported by Hayward and co-workers (Hayward et al. 2001 ). [springerplus.springeropen.com]

• Pathologist

  Both pathologists reached the same diagnosis and were unable to identify the primary site. The final diagnosis was melanoma metastasis from an occult primary cancer. Fig. 3. Necropsy of the brain and lungs. [karger.com]

• Vomiting

  Speech difficulties (slurring, hard to understand, inability to speak) Decreased muscle tone Nausea or vomiting OMAS may be caused by an immune reaction to a tumor called neuroblastoma or an immune reaction to a viral illness. [chop.edu]

  She was treated with clonazepam with gradual improvement of vomiting, headache, ataxia, and opsoclonus, but persistence of distal myoclonus in the upper limbs. [tr-ex.me]

  […] 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, vomiting [elsevier.es]

  She began to have nausea and vomiting a few days after the onset of dizziness. Subsequently, she noted unsteady gait and jerkiness of eye movements bilaterally. [file.scirp.org]

  • (3) behavioral changes or sleep disturbances, and • (4) diagnosis of neuroblastoma Additional signs on examination that may be suggestive of neuroblastoma include periorbital ecchymoses (“raccoon eyes”) In adults: dizziness, imbalance, nausea, and vomiting [slideshare.net]

• Nausea

  Speech difficulties (slurring, hard to understand, inability to speak) Decreased muscle tone Nausea or vomiting OMAS may be caused by an immune reaction to a tumor called neuroblastoma or an immune reaction to a viral illness. [chop.edu]

  She began to have nausea and vomiting a few days after the onset of dizziness. Subsequently, she noted unsteady gait and jerkiness of eye movements bilaterally. [file.scirp.org]

  […] myoclonus, • (3) behavioral changes or sleep disturbances, and • (4) diagnosis of neuroblastoma Additional signs on examination that may be suggestive of neuroblastoma include periorbital ecchymoses (“raccoon eyes”) In adults: dizziness, imbalance, nausea [slideshare.net]

  The fever was associated with headache and myalgia and she did not have nausea, vomiting, or skin rashes. On admission to our hospital she was afebrile and nuchal rigidity was present. She was conscious but aphasic. [jmedicalcasereports.biomedcentral.com]

  Nausea and vomiting were present in 12 patients, altered sensorium in 7, and seizures in 2. [annalsofian.org]

• Fecal 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]

• Strabismus

  J Pediatr Ophthalmol Strabismus. 2010 Jul 22. 47 Online:e1-3. [Medline]. American Academy of Ophthalmology. Basic and Clinical Science Course. Neuro-ophthalmology. 1999-2000. 5: 139-41. American Academy of Ophthalmology. [medscape.com]

  Besides these classic findings, irritability, headache, malaise, visual difficulty, dysphasia, mutism, strabismus, vomiting, drooling, lethargy, and sleep disturbances are also present. [scielo.br]

• Abnormal Behavior

  Opsoclonus-myoclonus-ataxia syndrome (often referred to as OMAS or opsoclonus-myoclonus syndrome) is an autoimmune disorder of the nervous system characterized by new movements of the limbs and eyes, abnormal behaviors, sleep dysregulation, and difficulty [chop.edu]

  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]

• Behavior Disorder

  Opsoclonus Myoclonus Syndrome (OMS) is a rare condition in children, that for expert consensus is defined to be met three of the four criteria: opsoclonus, ataxia, myoclonus, behavioral disorders or sleep and diagnosis of neuroblastoma. [scielo.org.co]

• Myoclonus

  Opsoclonus myoclonus syndrome: Response to plasmapheresis. Indian Pediatr 2004;41:499-502. 22. Srivastava T, Thussu A. Palatal myoclonus in postinfectious opsoclonus myoclonus syndrome: A case report. Neurol India 1999;47:133. 23. [neurologyindia.com]

  Because of these characteristics, opsoclonus is also often referred to as saccadomania. • Myoclonus is a rapid, abnormal movement featuring brief, shock-like muscle contractions (“positive myoclonus”) or muscle tone inhibitions (“negative myoclonus”) [slideshare.net]

  Paraneoplastic opsoclonus myoclonus had a good outcome in our experience. [pediatricneurosciences.com]

  S My son has a rare disorder called opsoclonus myoclonus. [medical-dictionary.thefreedictionary.com]

  myoclonus syndrome associated with cytomegalovirus encephalitis (see... [aan.com]

• Ataxia

  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]

  Frequently, generalised myoclonus and ataxia coexist; hence, the term opsoclonus-myoclonus-ataxia. [casereports.bmj.com]

  Opsoclonus - Myoclonus - Ataxia associated Neuroblastoma Neuroblastoma with Paraneoplastic syndrome (Opsoclonus-Myoclonus-Ataxia) Extensive lymphocytic infiltration with follicular formation. [spponline.org]

  O psoclonus-myoclonus ataxia syndrome is a paraneoplastic syndrome observed in 2% to 3% of children with neuroblastoma. 1 Children with opsoclonus-myoclonus ataxia syndrome present acutely with myoclonus, ataxia, and oculomotor symptoms and then rapidly [neuro.psychiatryonline.org]

  Acute cerebellar ataxia acute cerebellitis and opsoclonus-myoclonus syndrome. J Child Neurol. 2012; 27:1482-1488. Sivaswamy L. Approach to acute ataxia in childhood: diagnosis and evaluation. Pediatr Ann. 2014; 43:153-159. [slideshare.net]

• Irritability

  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]

  Parents noted that subsequent steroid therapy seemed to worsen irritability, lability and aggression, although motor symptoms improved. [neuro.psychiatryonline.org]

  […] attending.T.H., a 13-month-old boy, was admitted to our hospital with a progressive 6-week history of "twitchy and jerky" movements of the face, lips, tongue, trunk and upper extremities; in addition, there had been progressive loss of head control, irritability [nejm.org]

  The occurrence of marked irritability with behavioural change and sleep disturbance in an infant with new onset ataxia is one of the strongest clues that this may be OMS/DES. [gosh.nhs.uk]

  A decrease in muscle tone, lethargy, irritability, and malaise (a vague feeling of bodily discomfort) may also be present. Opsoclonus myoclonus may occur in association with tumors or viral infections. It is often seen in children with tumors. [childneurologyfoundation.org]

• Tremor

  Between the horizontal tremors which always predominate, sudden jolts appear occurring in other directions. [jneurology.com]

  Next came the occasional uncharacteristic missteps and falls — she’d been walking since before age one — and then the weird hand tremors that sent Keira’s parents scrambling to the hospital with no idea what was wrong. [globalgenes.org]

  Discussion Viral encephalitis classically causes fever, headache, altered sensorium, neuropsychiatric manifestations, catatonia, and abnormal movements such as orolingual tremors, myokymia, and lip smacking, and mandibulo-faciolingual tremors are also [jmedicalcasereports.biomedcentral.com]

  The frequency of the tremor is typically high, more than 4–5 Hz, whereas in our two cases the polygrafic EEG did not show the characteristic high frequency pattern. Furthermore, the tremor did not stop while the limbs were repositioned. [ccrejournal.eu]

  The main symptom is represented by distinct changes in coordination, tremor, myoclonus, specific movements of the eyeballs (opsoklonus), aggressive behavior, sleep disorders, and cognitive functions. [posterng.netkey.at]

• Cerebellar Ataxia

  This condition was characterized by the subacute onset of polymioclonia, cerebellar ataxia and opsoclonus that set later, following an herpes zoster infection. Steroid therapy resulted in rapid dramatic improvement of neurological symptoms. [scielo.br]

  Acute cerebellar ataxia represents cerebellar inflammation that typically occurs following infection or immunization. [aao.org]

  Orzechowski, in fact, identified in this paper dysmetria and other signs of cerebellar dysfunction, not ocular dysmetria or ocular ataxia in a 21-year-old female. [jneurology.com]

  The “opso-“ refers to the presence of intermittent jerky eye movements (dancing eyes) and the “myoclonus” to myoclonic jerks and cerebellar ataxia (dancing feet). [radiopaedia.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]

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]

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]

If neuroblastoma is found in a patient with opsomyoclonus, it is generally small with relatively favourable histology and the prognosis, in terms of survival, is very good. [radiopaedia.org]

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]

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]

Among paraneoplastic etiologies in adults with OMS, small cell lung cancer is the most common. While the pathophysiology is unknown, OMS is felt to be related to autoimmunity. [radiopaedia.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]

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]

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]

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]

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]

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]

[…] 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]

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]

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]

40th Interscience Conference on Antimicrobial Agents and Chemotherapy 2000 Sep 17; 316 Definition: serious = an event when the patient outcome is death, life-threatening, hospitalisation, disability, congenital anomaly, or requires intervention to prevent [medscape.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]