Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease in childhood which can be associated with neuroblastoma. Since autoantibodies have been detected in some patients with OMS, an autoimmune etiology is suspected. We compared the prevalence of autoimmune disorders and autoantibodies in parents of children with OMS and in a group of controls of same age and sex. Autoimmune diseases were found in 15.8% of the parents of OMS children, but only in 2.0% of the controls (p<0.001) There was also an increased prevalence of autoantibodies in the OMS parents (42.8% vs. 8.0%, p<0.001). Thyroid diseases were the most frequent autoimmune diseases found, followed by inflammatory rheumatic diseases. Interestingly, the OMS parents also had significantly more autoantibodies against CNS structures than the controls (p<0.01). These findings support the autoimmune hypothesis of childhood OMS and may also hint to a genetic susceptibility for OMS.
 ...
PMID:Increased prevalence of autoimmune disorders and autoantibodies in parents of children with opsoclonus-myoclonus syndrome (OMS). 1798 58

Opsoclonus-myoclonus syndrome is a rare paraneoplastic disorder, often associated with malignancies including neuroblastoma (NB), the most common solid tumor of childhood derived from the sympathetic nervous system. The pathogenesis of this syndrome is still undefined but is suspected to be the result of an autoimmune response. In this respect, different autoantibodies binding to neurons or cerebellar Purkinje cells have been detected in OMS-associated NB. In addition, immunohistochemical analysis of NB affecting children with OMS demonstrated the presence of interstitial or perivascular lymphoid infiltrates resembling secondary lymphoid follicles. Immunophenotyping of these lymphoid cells showed the existence of a mesh of CD21(+) follicular dendritic cells, numerous CD20(+) B lymphocytes in the germinal center and the mantle zone of the follicle and few CD3(+) T lymphocytes in a perifollicular area. Since information is lacking about the mechanism involved in ectopic lymphoid neogenesis of OMS-associated NB, we are currently investigating the role of different chemokines and their cognate receptors in the recruitment of lymphoid cells within tumor mass of OMS-associated NB. Here, we review some recent data about the pathogenesis of OMS-associated with NB.
 ...
PMID:Immunological mechanisms in opsoclonus-myoclonus associated neuroblastoma. 1857 42

Opsoclonus-myoclonus syndrome or Dancing Eye Syndrome (OMS/DES) is a rare neurological disorder of children, which associates with neuroblastoma (NB) in approximately 50% of cases. We examined sera from five patients with (OMS-NB(+)) and five without NB (OMS-NB(-)) for autoantibodies. OMS-NB(-) IgG bound to the surface of a NB cell line, whereas IgG from OMS-NB(+) and from NB patients without OMS/DES bound only to permeabilised cells. Both OMS-NB(+) and OMS-NB(-) reduced proliferation of NB cells. We also present a case report of a child with OMS/DES without NB who made a complete recovery without treatment. Serum antibodies at presentation bound to the surface and decreased NB cell proliferation but had decreased 9 weeks later when the child was asymptomatic. These results demonstrate that sera from some OMS/DES patients contain IgG antibodies that are potentially pathogenic.
 ...
PMID:Autoantibodies in childhood opsoclonus-myoclonus syndrome. 1868 75

The incidence, mode of presentation and management of Dancing Eye Syndrome/Opsoclonus-Myoclonus Syndrome (DES/OMS) was prospectively evaluated in 20 United Kingdom (UK) paediatric neurology centres by questionnaire over a 24-month period between 2003 and 2005. Nineteen children were notified, giving an incidence of 0.18 cases per million total population per year. Mean age at presentation was 18 months (range 3-42 months). Fifteen families consented to participate in the study. Atypical features were present in 6/15 cases including very delayed presentation of opsoclonus, dysphagia, and rapid spontaneous improvement without treatment. Only 4/15 cases were associated with neuroblastoma (NB) but current practice in excluding this is diverse and a standardised approach is suggested.
 ...
PMID:A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom. 1942 68

Opsoclonus-myoclonus syndrome (OMS) is a rare acquired movement disorder occurring in all age groups, predominantly in infants. Although the exact pathogenesis is still undefined, there is strong evidence for a paraneoplastic or parainfectious immune process resulting in central nervous system dysfunction. Mycoplasma pneumoniae has been implicated in a number of immune-mediated neurologic diseases [28]. However, the association of M. pneumoniae and opsoclonus-myoclonus-ataxia syndrome is not well established so far. We present three cases with opsoclonus-myoclonus-ataxia syndrome in adolescents following an infection with M. pneumoniae. Monophasic disease course and full recovery correspond to the favorable prognosis known from parainfectious cases in young adults. This should affect therapeutic consideration. OMS should be added to the spectrum of M. pneumoniae-associated neurologic complications. Nevertheless, neuroblastoma has to be ruled out in all cases of OMS.
 ...
PMID:Mycoplasma pneumoniae associated opsoclonus-myoclonus syndrome in three cases. 1994 62

Opsoclonus-myoclonus syndrome (OMS) is a rare condition that has been reported from all parts of the world. It is well recognized as a paraneoplastic syndrome in children with neuroblastoma and in adults with small-cell carcinoma of lung and some other cancers. It may also occur in association with various central nervous system infections. We report a case of OMS in a patient with varicella zoster virus infection. IgM antibody for varicella-zoster virus was detected in the serum and the cerebrospinal fluid. The patient improved after treatment with clonazepam and was asymptomatic at 1-month follow-up.
 ...
PMID:Opsoclonus-myoclonus syndrome caused by varicella-zoster virus. 2108 35

Opsoclonus-myoclonus syndrome (OMS) is seen in 2-3% of children with neuroblastoma and is believed to be caused by an autoimmune process elicited by the tumor. Although long-term neurologic sequelae are common in children with OMS, limbic encephalitis has not previously been reported. We report a child who developed limbic encephalitis associated with anti-Hu antibodies, 6 years after her initial diagnosis of neuroblastoma and OMS. This case demonstrates that patients with neuroblastoma and OMS are at risk for developing new paraneoplastic symptoms years after their original diagnosis and emphasizes the need for careful long-term follow-up.
 ...
PMID:Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma. 2148 Apr 75

We present a case of pediatric primary cervical neuroblastoma (NB), which is extremely rare. A 3-year-old girl with ataxia but no nasal obstruction, dysphagia, or stridor was diagnosed with cervical NB. Diagnostic results including clinical chemistry, full blood count, and serology, were normal. Ataxia worsened within a few days after onset and was not cured by steroids or intravenous immunoglobulin, delaying a definite diagnosis until a tumor was detected. Opsoclonus-myoclonus syndrome is typified by opsoclonus with myoclonus and ataxia, primarily associated with neuroblastoma. Brain stem cell and cerebellum dysfunction is thought to be due to an autoimmune mechanism. Following chemotherapy and selective neck dissection, the girl has had no recurrence or adverse sequelae. Ataxia disappeared during chemotherapy. We suggest that neuroblastoma should be considered in any child with unexpected by prolonged ataxia.
 ...
PMID:[A case report on cervical neuroblastoma with ataxia]. 2170 71

Opsoclonus-myoclonus syndrome, also named Myoclonic Encephalopathy of Infants, Opsoclonus- Myoclonus Ataxia, Dancing Eyes - Dancing Feet Syndrome, Dancing Eyes Syndrome, Kinsbourne syndrome, is a rare, paraneoplastic or possibly post-viral chronic neurological disorder. The age of presentation ranges from 6 months to 3 years. In 50% of affected children the syndrome is associated with an underlying occult or clinically apparent neuroblastoma. In most patients the tumour is localized, small and well differentiated, with no NMYC gene copy number amplification. The syndrome may also occur after tumour resection or at relapse. The opsoclonus-myoclonus syndrome can occur in children without neuroblastoma, in such idiopathiccases, the onset of neurological symptoms is related to infection. It is assumed, that in idiopathic cases the syndrome could have developed in the course of neuroblastoma which had undergone a complete spontaneous regression. The most characteristic clinical features of opsoclonus-myoclonus syndrome are: opsoclonus, myoclonus, ataxia, irritability, mutism and sleep disturbances. The disease course is usually long-term with episodes of remission and relapses. Approximately 80% of children with opsoclonus-myoclonus syndrome suffer from mild to severe neurological handicaps, mainly cognitive impairment. The authors present a 2-year old boy with opsoclonus-myoclonus syndrome preceded by involution of prenatally documented retroperitoneal area tumour.
 ...
PMID:[Opsoclonus-myoclonus syndrome in a 2 year old boy with prenatally diagnosed retroperitoneal tumour]. 2200 47

Opsoclonus-myoclonus syndrome is a rare neurological disorder. In children, the etiology varies, although it is a paraneoplastic manifestation (mainly of neuroblastoma) in 40% to 80% of cases. Whole-body MRI promises to be a powerful tool in the search for a possible primary tumor in this condition for which the diagnostic algorithm is yet to be established. We present the case of a two-year-old boy with signs of opsoclonus-myoclonus syndrome in whom a retroperitoneal neuroblastoma was detected by whole-body MRI.
...
PMID:[Whole-body magnetic resonance imaging in a patient with an occult abdominal neuroblastoma and opsoclonus-myoclonus syndrome]. 2281 98


<< Previous 1 2 3 Next >>