It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. In children, most cases are associated with neuroblastoma and most of the others are suspected to be associated with a low-grade neuroblastoma that spontaneously regressed before detection. In adults, most cases are associated with breast carcinoma or small-cell lung carcinoma. OMS is not dan duchaine pdf download on genetically.
After a diagnosis of OMS is made, an associated neuroblastoma is discovered in half of cases, with median delay of 3 months. Unsourced material may be challenged and removed. There is no known definitive cure for OMS. However, several drugs have proven to be effective in its treatment. Subsequent very gradual tapering with pills generally follows. Most patients require high doses for months to years before tapering. Is known to stimulate the immune system.
Currently there are no clinically established laboratory investigations available to predict prognosis or therapeutic response. Tumors in children who develop OMS tend to be more mature, showing favorable histology and absence of n-myc oncogene amplification than similar tumors in children without symptoms of OMS. Involvement of local lymph nodes is common, but these children rarely have distant metastases and their prognosis, in terms of direct morbidity and mortality effects from the tumor, is excellent. Although the symptoms of OMS are typically steroid-responsive and recovery from acute symptoms of OMS can be quite good, children often suffer lifelong neurologic sequelae that impair motor, cognitive, language, and behavioral development. Most children will experience a relapsing form of OMS, though a minority will have a monophasic course and may be more likely to recover without residual deficits. Viral infection may play a role in the reactivation of disease in some patients who had previously experienced remission, possibly by expanding the memory B cell population.
OMS will have long-term neurologic, cognitive, behavioral, developmental, and academic impairment. Since neurologic and developmental difficulties have not been reported as a consequence of neuroblastoma or its treatment, it is thought that these are exclusively due to the immune mechanism underlying OMS. Patients with OMA and neuroblastoma have excellent survival but a high risk of neurologic sequelae. Favourable disease stage correlates with a higher risk for development of neurologic sequelae. The role of anti-neuronal antibodies in late sequelae of OMA needs further clarification”. Residual behavioral, language, and cognitive problems occurred in the majority”. These studies are focused on finding ways to prevent, treat, and cure these disorders, as well as increasing knowledge about them.
Opsoclonus-myoclonus syndrome: A clinicopathological confrontation”. Opsoclonus-myoclonus associated with celiac disease”. The neurobiology of the opsoclonus-myoclonus syndrome”. Clinical Pediatric Neurology, 3rd edition, 2009, page 205. Immunologic and clinical responses to rituximab in a child with opsoclonus-myoclonus syndrome”. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: histopathologic features-a report from the Children’s Cancer Group”.
Ataxia and secretory diarrhea: two unusual paraneoplastic syndromes occurring concurrently in the same patient with ganglioneuroblastoma”. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies-a report from the Children’s Cancer Group Study”. Adaptive control of saccades in children with dancing eye syndrome”. Longitudinal neurodevelopmental evaluation of children with opsoclonus-ataxia”.
Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis”. Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies”. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae”. B- and T-cell markers in opsoclonus-myoclonus syndrome: immunophenotyping of CSF lymphocytes”. The association between neuroblastoma and opsoclonus-myoclonus syndrome: a historical review”. Laurie-Ann Duchaine Foundation: a non-profit foundation dedicated to funding to help families, find more cases in the world.