Presentation
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]
Entire Body System
- Asymptomatic
It usually becomes chronic, with relapses varying in number and intensity. 6 Our patient, however, remained asymptomatic and displayed no short-term sequelae after 24 months of treatment. [elsevier.es]
A majority of infections are asymptomatic and only less than 1% manifest as encephalitis [ 1 ]. [jmedicalcasereports.biomedcentral.com]
After follow-up of 24 months, the child was asymptomatic, but CT of the abdomen revealed mass of same size that was documented 2 years back. One child (case 3) died of febrile encephalopathy (not related to disease). [pediatricneurosciences.com]
Respiratoric
- Pertussis
[…] immunodeficiency virus, and hepatitis C. 12, 15-16 There are also reports of antecedent bacterial infections including Mycoplasma pneumoniae and salmonella. 12, 16 Post-immunization associations have been reported following varicella, measles, and diphtheria-pertussis-tetanus [aao.org]
Gastrointestinal
Cardiovascular
- Hypertension
Over time, treatment with ACTH may have substantial cortisol-related adverse effects that must be monitored carefully, particularly weight gain, hypertension, and reductions in bone density. [rarediseases.org]
Psychiatrical
- 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]
Neurologic
- 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]
- Involuntary Movements
movements and neurologic damage. [bbb.sas.upenn.edu]
Generalized action-myoclonus and the opsoclonus, a distinct ocular dyskinesia consisting of quick, involuntary movements of the eyeballs are the hallmarks of the disease. We describe the clinical course of a 63 year old man from our institution. [deepdyve.com]
movement Mycoplasmataceae Mycoplasmatales Eye disease Nervous system diseases Oculomotor syndrome Neurological disorder Keyword (es) Asociación Niño Infección Mycoplasma pneumoniae Mioclonia Opsoclonación Pediatría Salud pública Bacteria Hombre Mollicutes [pascal-francis.inist.fr]
Neurologic examination revealed spontaneous, involuntary, arrhythmic and conjugate rapid eye movements; facial, axial and appendicular myoclonus and gait ataxia. Her motor strength and deep tendon reflexes were normal. [academic.oup.com]
- Vertigo
Acute vertigo with vomiting and/or gait instability was the most common symptom presentation in I-OMS or P-OMS, often leading to an initial misdiagnosis of peripheral vertigo. [ncbi.nlm.nih.gov]
The patient had a sudden-onset disabling holocranial headache, aggressive behavior, and vertigo for the 2 weeks prior to her arrival at the Emergency Department. [karger.com]
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]
- Insomnia
Symptoms of OMAS include: Rapid, involuntary eye movements (opsoclonus) Brief, involuntary twitching of muscle (myoclonus) Loss of muscle control or coordination of movements (ataxia) Unbalanced walk (unsteady gait) Sleep disturbances (insomnia) Changes [chop.edu]
Behavioral and sleep disturbances, including extreme irritability, inconsolable crying, reduced and fragmented sleep (insomnia) and rage attacks are common. [rarediseases.org]
He also had insomnia, major depressive symptoms, and mild cognitive deficits. Findings on magnetic resonance imaging of the brain and cervical spine as well as electroencephalography were normal. [ncbi.nlm.nih.gov]
- Abnormal Gait
gait and sudden repetitive jerky limbs' movements. [casereports.bmj.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]
Treatment
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
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]
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]
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]
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]
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]
Pathophysiology
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]
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]
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]