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)

Opsoclonus-myoclonus ataxia syndrome is a paraneoplastic syndrome of cerebellar damage associated with neuroblastoma. The authors assessed psychiatric symptoms of opsoclonus-myoclonus ataxia syndrome in 17 children, who were 16 months to 12(1/2) years of age. Psychiatric symptoms examined included disruptive behavior, affective dysregulation, irritability, impulsivity, cognitive impairment, and poor attention.
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PMID:Mood and behavioral dysfunction with opsoclonus-myoclonus ataxia. 1672 Aug 3

Opsoclonus-myoclonus, a rare paraneoplastic syndrome that may occur in patients with neuroblastoma, is thought to be a humorally mediated immune reaction to malignant cells that cross-react with autoantigens. This report describes the case of an occult neuroblastoma diagnosed in a 4-year-old female 2 years after presentation of opsoclonus-myoclonus. Although no mass was evident on previous imaging at an interval of 10 months, a computed tomographic scan 4 months after rituximab treatment and 20 months after presentation revealed a new left adrenal mass. Although neuroblastomas can be identified months after presentation of opsoclonus-myoclonus without treatment with rituximab, this report describes one of the longest intervals using up-to-date imaging techniques. Therefore the case raises two concerns: (1) whether the same immune process that causes opsoclonus-myoclonus may suppress neuroblastomas, and (2) whether immunosuppressive therapy with rituximab may inhibit the immune reaction to occult neuroblastomas in patients with unexplained opsoclonus-myoclonus.
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PMID:Neuroblastoma found in a 4-year-old after rituximab therapy for opsoclonus-myoclonus. 1693 63

Opsoclonus-myoclonus-ataxia-syndrome (OMS) represents a rare neuroblastoma-associated paraneoplastic syndrome that commonly results in neurologic deficits despite tumor resection and immunosuppressive therapy. We describe the response of five such children to high-dose dexamethasone pulses including two patients in whom previous glucocorticoids, rituximab, and cytostatic drugs were not successful. All patients had MYCN non-amplified tumors that were detected 1 to 7 months after the onset of the OMS or ataxia. This treatment resulted in a good partial response in three and in complete remission in two patients. Our results show that dexamethasone pulses are likely to be useful for both, first-line- and salvage-therapy for OMS-patients.
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PMID:Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses. 1722 43

Opsoclonus-myoclonus-ataxia syndrome is a rare disorder frequently associated with neuroblastoma in pediatric patients. We present the case of an 18-month-old child with symptoms classic of the syndrome and review the current literature with the aim of aiding the clinician in the recognition and early management of this disease.
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PMID:Dancing eyes, dancing feet: opsoclonus-myoclonus in an 18-month-old child with neuroblastoma. 1809 97

Opsoclonus-myoclonus-ataxia (OMA) syndrome is a rare neurobehavioral paraneoplastic disorder in children with neuroblastic tumors. The neurologic symptoms are generally treated with a number of immunosupressive and immunomodulating agents. A 4-year-old previously healthy male patient was admitted to the authors' center with progressive ataxia, gait disturbance, difficulty of speech, and opsoclonus. He had a diagnosis of ganglionueroblastoma at the thoracal paraspinal region. Following surgery, the patient received IVIG and prednisolone but his cerebellar symptoms progressed. Rituximab therapy was started and continued for total 8 weeks without any side effect. The authors observed excellent neurologic response in the patient at the 4th week of treatment. Rituximab is a new, promising, and safe therapy for OMA syndrome in children with neuroblastoma.
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PMID:Response to rituximab and prednisolone for opsoclonus-myoclonus-ataxia syndrome in a child with ganglioneuroblastoma. 1906 42

The aim of this article is to review the paraneoplastic opsoclonus-myoclonus syndrome (POMS). Opsoclonus is characterized by involuntary, arrhythmic, chaotic, multi-directional saccades with horizontal, vertical and torsional components, and it is commonly accompanied by cerebellar ataxia and myoclonic jerks in the trunk and limbs. Parainfectious brainstem encephalitis, toxic-metabolic disturbances and others condition should be considered as potential causes of these symptoms. In adults, POMS is most commonly associated with small-cell lung cancer, breast cancer, and ovarian cancer. In children, a neuroblastoma is detected in approximately 50% of cases. Many autoantibodies have been detected in patients with POMS: this finding suggests the involvement of a humoral immune mechanism. However, most patients are seronegative for these autoantibodies. This implies that a cell-mediated immune mechanism may also be involved in the pathogenesis of opsoclonus. Although the exact pathophysiology mechanism of opsoclonus remains unclear, recent reports suggest that disinhibition of the fastigial nucleus of the cerebellum is involved. In children, the immunotherapy with corticosteroids, intravenous immunoglobulin, adrenocorticotropic hormone, plasma exchange, cyclophosphamide, or rituximab is used. Although opsoclonus is often responsive to therapy, the high incidence of sequelae related to motor function, speech, behavior, and sleep is an important problem. In adults, POMS is less responsive to immunotherapy and improves only with tumor resection. In order to develop novel and effective therapeutic strategies, further studies on the immunopathogenesis and pathophysiology of POMS are required.
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PMID:[Paraneoplastic opsoclonus-myoclonus syndrome--a review]. 2042 Jan 76

Opsoclonus-myoclonus-ataxia (OMA) syndrome is a rare neurological disorder, characterized by a rapid onset of generalized myoclonus in association with chaotic multi-directional eye movements and, less frequently, cerebellar ataxia. OMA is commonly related to a paraneoplastic process, specifically neuroblastoma in children and lung or breast cancer in adults. Nevertheless, OMA may occur in association with various infectious agents, such as Coxsackie virus B3, Epstein-Barr virus, mumps, enterovirus, and streptococcus. We recently encountered two cases of HIV-related OMA syndrome. The first patient developed a sudden onset of OMA at the time of HIV seroconversion. The second patient experienced severe ataxia with a mild degree of myoclonus and opsoclonus, associated with an elevated CD4 count following the initiation of highly active antiretroviral therapy (HAART). We suggest that OMA syndrome may be another rare manifestation of HIV infection at the time of seroconversion or during an immune restoration period.
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PMID:HIV-related opsoclonus-myoclonus-ataxia syndrome: report on two cases. 2042 23

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.
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PMID:[Opsoclonus-myoclonus syndrome in a 2 year old boy with prenatally diagnosed retroperitoneal tumour]. 2200 47

Opsoclonus myoclonus is a rare autoimmune condition characterized by cerebellar degeneration. It occurs most often as a paraneoplastic syndrome when a cancer remote to the brain induces cerebellar dysfunction that is unrelated to metastases. Half of all cases occur in children with a neuroblastoma. Most adults with opsoclonus myoclonus have neoplastic, infectious, metabolic, or idiopathic etiologies. Signs of cerebellar dysfunction noted at presentation include opsoclonus, myoclonus and ataxia, hence the name "dancing eyes, dancing feet syndrome." Opsoclonus is characterized by rapid, involuntary eye movements that are dysrhythmic and uncoordinated.Neuronal damage is induced by antibodies usually related to the primary pathology. Treatment targets the etiology and also employs steroids, plasmapheresis, immunosuppressive agents, or other anti-inflammatory therapies. Children with opsoclonus myoclonus resulting from a neuroblastoma often retain neurological sequelae. Adult cases of opsoclonus myoclonus with idiopathic or infectious etiologies have a more favorable prognosis than those with neoplastic origins.
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PMID:Opsoclonus myoclonus. 2224 16

Opsoclonus-myoclonus ataxia (OMA) syndrome is the most common paraneoplastic neurological syndrome of childhood, associated with occult neuroblastoma in 20%-50% of all cases. OMA is the initial presentation of neuroblastoma in 1%-3% of children. Conventional radiological imaging approaches include chest radiography and abdominal computed tomography (CT). Nuclear medicine techniques, in form of (123)I/(131)I-metaiodobenzylguanidine (MIBG) scintigraphy have been incorporated in various diagnostic algorithms for evaluation of OMA. We describe use of somatostatin receptor PET/CT with (68)Gallium- DOTA-DPhe(1), Tyr(3)-octreotate (DOTATATE) in diagnosis of neuroblastoma in two cases of OMA.
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PMID:Somatostatin receptor positron emission tomography/computed tomography (PET/CT) in the evaluation of opsoclonus-myoclonus ataxia syndrome. 2416 18


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