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

Neuroblastoma, a common tumor of neural crest origin, demonstrates an extremely variable clinical presentation and behavior. Paraspinal tumors may occur anywhere along the spinal column where they may invade the intervertebral foramina and produce cord compression. Cervical involvement may produce Horner's syndrome. Opsoclonus-myoclonus syndrome is also a well-known neuroimmunologic finding of neuroblastoma. We report a 28-month-old female presenting with ptosis and muscle weakness, diagnosed as having a neuroblastoma.
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PMID:Neuroblastoma: an unusual presentation with bilateral ptosis. 1508 9

Opsoclonus-myoclonus syndrome (OMS) is a rare, autoimmune neurological disorder that is poorly recognized and undertreated. Neuroblastoma is found in one half of the cases. Because of the high incidence of spontaneous regression of neuroblastoma, it is unknown whether not finding a tumor means there was none. To define demographic trends and the standard of care in the first large series of OMS, 105 children were recruited over a 13-year period in a retrospective questionnaire survey. Children with and without a tumor differed little in viral-like prodrome and neurological symptoms. Earliest neurological symptoms were staggering and falling, leading to a misdiagnosis of acute cerebellitis. Later symptoms included body jerks, drooling, refusal to walk or sit, speech problems, decreased muscle tone, opsoclonus, and inability to sleep. Tumor resection alone did not provide adequate therapy for most. Adrenocorticotropic hormone (ACTH), prednisone, and intravenous immunoglobulin were used with equal frequency, but ACTH was associated with the best early response. More than one half of the children had relapses. Residual behavioral, language, and cognitive problems occurred in the majority. The delay in diagnosis (11 weeks) and initiation of treatment (17 weeks) is unacceptably long.
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PMID:Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus-myoclonus syndrome. 1557 22

Opsoclonus-myoclonus syndrome (OMS) is a rare neurologic syndrome that, in children, associates with neuroblastoma in more than half of the cases. The etiology of this condition is thought to be immune mediated, but, though immunosuppressive therapies may ameliorate the acute symptoms, no effective treatment to prevent the common neuropsychologic sequelae has been established. This paper summarizes the results obtained at the 2004 Advances in Neuroblastoma Research meeting, providing status of the art information on immune pathogenesis, clinical features, acute and chronic neurologic manifestations, current and novel therapeutic approaches. It is emphasized that, due to the rarity of OMS in general and neuroblastoma-associated OMS in particular, international collaborations are needed to better define the pathogenesis and therapy of this disease, propose common evaluation criteria and identify new treatment modalities.
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PMID:Opsoclonus myoclonus syndrome in neuroblastoma a report from a workshop on the dancing eyes syndrome at the advances in neuroblastoma meeting in Genoa, Italy, 2004. 1592 8

Opsoclonus-myoclonus syndrome is a distinct neurologic disorder characterized by opsoclonic eye movements, multifocal myoclonus, and ataxia, traditionally described as "dancing eyes, dancing feet." A presenting sign in 2% of children with neuroblastoma, it usually heralds a favorable prognosis for the tumor. Although opsoclonus-myoclonus syndrome usually presents at initial diagnosis or relapse, there are reports of delayed presentation, usually a few months after diagnosis. This report describes a patient with ganglioneuroblastoma who developed recurrent symptoms of opsoclonus-myoclonus syndrome 9 years after completing treatment, without evidence of recurrent tumor. Believed to be autoimmune in origin, opsoclonus-myoclonus syndrome frequently responds to immunomodulatory therapies, such as steroids or intravenous immunoglobulin. This patient did not respond adequately to either agent, so plasmapheresis, a less commonly used modality in opsoclonus-myoclonus syndrome, was attempted. His symptoms resolved after he received therapy with a combination of plasmapheresis and steroids over a 1-year period. After being slowly weaned off all therapy, he has been symptom-free now for over 3 years. Armstrong MB, Robertson PL, Castle VP. Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis.
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PMID:Delayed, recurrent opsoclonus-myoclonus syndrome responding to plasmapheresis. 1624 25

Opsoclonus-myoclonus syndrome (OMS) is a rare paraneoplastic syndrome that occurs in 2%-3% of patients with neuroblastoma. The cause of this syndrome is believed to be immune mediated, but the exact mechanism still remains unclear. There is an urgent need to improve our current strategies for treating patients with OMS, as many patients have significant long-term neurologic deficits and behavior disorders with current treatment approaches. Therapies that have shown to improve symptoms in these patients have ranged from ACTH and corticosteroids, to intravenous gammaglobulin and plasmapheresis. We report our experience with Rituximab in a patient with neuroblastoma and OMS.
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PMID:Rituximab for treatment of opsoclonus-myoclonus syndrome in neuroblastoma. 1690 Apr 84

Opsoclonus-myoclonus syndrome (OMS) in children is a rare neurological condition with opsoclonus, myoclonus, ataxia and irritability in the first 2 years of life. It can be idiopathic, parainfectious, or paraneoplastic with tumours of the neural crest. Few studies of long-term follow-up after OMS have been published. We investigated the motor, cognitive and behavioural outcome of ten patients (eight girls and two boys) seen between 1987 and 2002. We reviewed the records and reassessed the patients. A ganglioneuroma was found in one patient and a neuroblastoma in another. Tumour resection did not influence the OMS. The age at diagnosis was 10-24 months and the follow-up period 1-17 years (average 6.5 years). The interval between the first signs and symptoms and starting treatment was 2-12 weeks: treatment consisted of different immunosupressants. Remission was achieved within 5 months in seven, and relapses were present in seven of ten. At follow-up, only one child had mild ataxia. IQ testing was performed in nine with scores below 75 in four and above 85 in four. Attention deficit and visuomotor difficulties led to school problems with special needs, also in those three children with normal IQs. Only two children were attending regular schools. Behavioural problems were reported in seven, and speech difficulties were present in five. In conclusion, the long-term outcome in our patients with OMS was dominated by cognitive and behavioural problems and not by ataxia. Compared with previous reports, our patients were treated earlier. Larger studies and uniform treatment protocols are needed to demonstrate whether early and prolonged immunosupressant therapy has a favourable influence on outcome.
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PMID:Long-term outcome of ten children with opsoclonus-myoclonus syndrome. 1708 89

Opsoclonus-myoclonus syndrome (OMS) is a rare movement disorder characterized by chaotic eye movements, myoclonus, and ataxia associated with severe irritability. Different treatment modalities including steroids and cyclophosphamide have been tried in the past often with significant side effects and variable success. Here we present 11 children, diagnosed with OMS between 1999 and 2005 and treated with high dose dexamethasone pulses. Main symptoms at presentation were opsoclonus (11/11), ataxia and/or myoclonus (11/11), irritability (10/11) associated with a neuroblastoma in four children. Number of dexamethasone pulses ranged from 6 to 60 pulses. No major side effects were reported. In 6/11 children a complete and sustained remission of OMS symptoms was achieved after 6 to 29 pulses of dexamethasone. Two children from this group have a normal development and no neurological sequelae. Two further children have minor delays in fine- and gross-motor skills. Two children despite a complete recovery of OMS symptoms have persisting developmental problems. 5/11 children still require regular dexamethasone pulses in addition to daily prednisolone (n = 1) or have received cyclophosphamide pulses meanwhile (n = 2). All children continue to have developmental and neurological difficulties. In summary treatment with high dose pulsatile dexamethasone appears to be safe and beneficial in a subgroup of patients with OMS.
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PMID:High dose pulsatile dexamethasone therapy in children with opsoclonus-myoclonus syndrome. 1723 8

Opsoclonus-myoclonus syndrome (OMS) in children is a rare disorder including a severe eye movement disturbance, myoclonia, ataxia and often developmental retardation. Both OMS forms, idiopathic or neuroblastoma-associated (paraneoplastic), have been suspected to be autoimmune. Recently, autoantibodies have been found in OMS sera. We here show that autoantibodies in OMS, both intracellular and surface binding, belong mainly to the IgG3 subclass, although the total serum IgG3 level is normal. These results support the autoimmune hypothesis and point to a protein autoantigen as antigenic target.
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PMID:IgG subclass distribution of autoantibodies in pediatric opsoclonus-myoclonus syndrome. 1732 72

Opsoclonus-myoclonus syndrome (OMS) is a rare and debilitating disorder of unknown etiology affecting children and adults. Outcome is unfavourable; approximately 80% of children with OMS suffer from mild to severe neurological handicaps, mainly cognitive impairment. A standard therapy does not exist. Due to the possible immune-mediated mechanisms, treatment with steroids, ACTH, plasmapheresis and immunoglobulins can be successful. However, some children become steroid dependent and symptoms may reoccur after treatment has been finished. We present two girls with OMS, who had a prolonged clinical course lasting 4 and 9 years with many relapses. Both children developed symptoms around the age of two years. Diagnostic work-up to exclude neuroblastoma was negative. Several treatment modalities including oral steroids, dexamethasone pulses, immunoglobulin and cyclosporine were used without lasting success. In addition, cognitive impairment developed in both children. In order to prevent further clinical and mental deterioration, 6 pulses of cyclophosphamide in combination with dexamethasone pulses every 4 weeks were administered. Both children showed significant improvement of OMS symptoms. One girl is still symptom free 18 months after treatment, mild ataxia developed in the other after 12 months. Both children are mentally handicapped and in special need schools. We conclude that combination of cyclophosphamide pulses and dexamethasone pulse therapy is a therapeutic option even after a long clinical course to improve symptoms of OMS.
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PMID:Chronic relapsing opsoclonus-myoclonus syndrome: combination of cyclophosphamide and dexamethasone pulses. 1762 38

Opsoclonus-myoclonus syndrome (OMS) is a rare neurologic disorder comprising the main symptoms of eye-movement disturbances, muscle jerks, and severe ataxia. In children and adults, some cases are associated with a tumor as a paraneoplastic syndrome, whereas in children the paraneoplastic form is almost exclusively associated with neuroblastoma. The detection of autoantibodies in some OMS sera led to the hypothesis that the syndrome is of autoimmune origin. Beside autoantibodies against intracellular proteins, such as anti-Hu, alpha-enolase, and KHSRP, specific binding of autoantibodies to the surface of neuroblastoma cells and cerebellar granular neurons have been found. Antiproliferative and proapoptotic effects of these autoantibodies on neuroblastoma cell lines were noted as well. These results support the concept of a humoral autoimmune process in the pathogenesis of OMS.
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PMID:New autoantibodies in pediatric opsoclonus myoclonus syndrome. 1791 40


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