Presentation
[…] for the paraneoplastic syndrome (4) a diagnosis of neuroblastoma.[3] In the adult population, OMS presents similarly, with symptoms of dizziness and loss of balance being the most likely presenting complaints.[4] Etiology The exact etiology for OMS remains [eyewiki.aao.org]
Upon arrival, she was haemodynamically stable, but presented tachycardia. [elsevier.es]
Our patient still had features of frontal lobe dysfunction 5 months after symptom presentation. [jamanetwork.com]
The patients present with truncal and limb ataxia, falls, opsocolunus and myoclonus. Also they could present a certain degree of encephalopathy, cerebelar damage signs and sleep disturbances. [medigraphic.com]
When there is a tumor present, treatment may include chemotherapy, surgery, and/or radiation. [rarediseases.info.nih.gov]
Entire Body System
- Malaise
[citation needed] Disease course and clinical subtypes[edit] In most cases OMS starts with an acute flare-up of physical symptoms within days or weeks, but some less obvious symptoms such as irritability and malaise may begin weeks or months earlier. [en.wikipedia.org]
His symptoms started with malaise, fever, myalgia, coughs, and 2 days later dyspnea. After nearly 4 days of symptoms’ onset, he developed generalized jerky movements. His voice and ocular movements were spared. [link.springer.com]
- Cushingoid
Side effect was monitored closely for Cushingoid feature, hypertension, headache, obesity, and infection. No significant side effects were noted. They were supplemented with calcium and Vitamin D. Salt restriction was followed. [journals.lww.com]
Neurologic
- Nystagmus
Unlike nystagmus, saccadic intrusions, including opsoclonus, do not contain a slow phase and consist entirely of rapid movements. [eyewiki.aao.org]
DIFFERENTIAL DIAGNOSIS • Square wave jerks, ocular flutter, nystagmus • Acute cerebellar ataxia/acute cerebellitis spectrum • Labyrinthitis • GBS • Epileptic seizures Desai J, Mitchell WG. [fr.slideshare.net]
Other names for OMS include:[citation needed] Dancing Eyes-Dancing Feet syndrome Dancing Eyes syndrome (see also Nystagmus) Kinsbourne syndrome Myoclonic Encephalopathy of Infants (MEI), not to be confused with Early myoclonic encephalopathy (EME) Opsoclonic [en.wikipedia.org]
- Motor Disturbances
[…] that can progress later in life.[11] Summary OMS is a rare ocular motor disturbance characterized by multivectorial, bilateral, conjugate back to back saccades without intersaccadic interval. [eyewiki.aao.org]
Workup
Immunologic workup revealed the presence of antibodies to NR1/NR2 heteromers of NMDAR, confirming the diagnosis of anti-NMDAR encephalitis. [jamanetwork.com]
Nevertheless, exclusion of primary central nervous system pathology through neuroimaging and cerebrospinal fluid analysis is typical as part of the diagnostic workup. [eyewiki.aao.org]
Microbiology
- Plasmodium Falciparum
Blood examination revealed mild pallor with falciparum tropozoite; Plasmodium falciparum antigen (Plasmodium lactate dehydrogenase [pLDH]) was positive. [journals.lww.com]
Other Pathologies
- Gliosis
Support for this theory comes from histopathological examination, which has revealed gliosis and inflammation in the cerebellar vermis of patients with opsoclonus.[7][8] Differential diagnosis Given its rarity and sometimes atypical presentation, OMS [eyewiki.aao.org]
Treatment
There are no official treatment recommendations for OMS. [rarediseases.info.nih.gov]
Occasionally children recover fully without treatment. Others respond to treatment partially or incompletely. The majority of children however, will have a chronic relapsing disease course and will require long-term immunosuppressive treatment. [gosh.nhs.uk]
Treatment for OMAS involves removal and treatment of a tumor, if one is found. [chop.edu]
Your child’s doctor may recommend immunotherapy as treatment for opsoclonus-myoclonus syndrome. Immunotherapy is a type of treatment that helps keep the body’s immune system from attacking the brain. [childrenshospital.org]
Prognosis
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 [fr.slideshare.net]
If neuroblastoma is found in a patient with opsomyoclonus, it is generally small with relatively favorable histology and the prognosis, in terms of survival, is very good. [radiopaedia.org]
Opsoclonus-myoclonus syndrome: a new era of improved prognosis? Pediatric Neurology. 2017; 72:65-69. https://doi.org/10.1016/j.pediatrneurol.2017.03.011 Armangué T, Sabater L, Torres-Vega E, et al. [medigraphic.com]
Pranzatelli Abstracts. for more details Prognosis[edit] Currently there are no clinically established laboratory investigations available to predict prognosis or therapeutic response. [en.wikipedia.org]
However, tumor resection does not always lead to neurologic improvement and patients may require recurring treatment to control their symptoms.[4][10] Prognosis Regardless of treatment, opsoclonus usually resolves. [eyewiki.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]
The exact etiology for OMS remains unclear. [eyewiki.aao.org]
Multifactorial analysis of opsoclonus-myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children. [medigraphic.com]
Regarding the etiologic classification of myoclonus, OMS is placed under symptomatic myoclonus category and etiologically categorized into idiopathic, paraneoplastic, infectious, and other subgroups (Caviness 2019; Caviness and Brown 2004). [link.springer.com]
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
OUTLINE: • Introduction • Pathophysiology • Epidemiology • Clinical presentation • Diagnostic criteria • Differential diagnosis • Etiology / risk factors • Clinical work-up / diagnostic • Management • Prognosis • Summary 3. [fr.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]
[…] varicella, measles, and diphtheria-pertussis-tetanus vaccine administration.[4][6] Also, OMS has been attributed to toxic or metabolite abnormalities including phenytoin overdose, hyperosmolar non-ketotic diabetic coma, and cocaine intoxication.[6] Epidemiology [eyewiki.aao.org]
We analysed clinical and epidemiological characteristics of the cases, the treatment used and the medium- to long-term outcomes (Table 1). [analesdepediatria.org]
Regarding movement disorder neurological symptoms in COVID-19, the semiology, pathophysiology, and epidemiology of each movement disorder are still naïve. In some studies, there are classifications for staging the COVID-19 disease course. [link.springer.com]
Pathophysiology
An autoimmune pathophysiology is suspected because childhood OMS patients have functionally active autoantibodies, proinflammatory changes in the cytokine network and immunotherapy responses. [tandfonline.com]
In children, the most common age of presentation is between 1 and 3 years.[5] In adults, the age of presentation can vary widely, with reports ranging from adolescence to the eighth decade of life.[4] Pathophysiology of opsoclonus Although the exact pathophysiology [eyewiki.aao.org]
We report seven cases of opsoclonus-myoclonus syndrome presumably para-infectious in nature and discuss their phenomenology, their possible pathophysiological relationship to COVID-19 and diagnostic and treatment strategy in each case. [preprints.org]
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. [fr.slideshare.net]
Prevention
Our goal with treating Kate with combination immune therapy including weekly infusions is prevent her from developing permanent cognitive impairment,” says ,” says Joanna Blackburn, MD, Kate’s neurologist. [luriechildrens.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]
To prevent relapse, monthly dose of 2 g/kg/day of IVIG was given. After 1 year, ACTH was stopped. [journals.lww.com]
On physical exam, she had disorganized high amplitude conjugate horizontal movements of her eyes which persisted with eye closure, severe truncal ataxia that prevented her from sitting up without support, and distinct abdominal myoclonus. [hindawi.com]