Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Opsoclonus is a rare disorder of the saccadic system, in which fixation is continuously interrupted by multivectorial, back-to-back saccades that at times can be seen only with an ophthalmoscope. To diagnose it reliably, eye movement recording is required. Opsoclonus may be a harbinger of an occult malignancy, though many cases are postinfectious, toxic-metabolic or idiopathic. The underlying malignancy is usually neural crest tumors in children and lung, breast, or gynecologic cancer in adults. Opsoclonus can be accompanied by myoclonus and ataxia. Concurrent appearance of oscillations affecting eyes and limbs suggests a common brainstem generator. Dysfunction of the glycinergic omnipause neurons in the nucleus raphe interpositus has been proposed. Autoantibodies against neural epitopes shared with a tumor are implicated in the pathogenesis of opsoclonus in paraneoplastic cases. Because of the association with malignancies, full oncological work-up is indicated in every case. Coexisting opsoclonus carries a relatively good prognosis for the cancer; however, the neurologic disability may remain even if the tumor has been arrested. New, potentially effective immunoadsorption therapy for opsoclonus is currently under investigation.
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PMID:Opsoclonus. 887 53

A 67-year-old lady with breast cancer developed diplopia, tinnitus, nausea and vertigo within 2 weeks, followed 2 months later by severe truncal ataxia. Opsoclonus was never observed. She had anti-Ri antibodies and improved substantially after tumor resection and radiation.
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PMID:[Reversible paraneoplastic cerebellar symptoms. An example of anti-Ri syndrome]. 967 76

After two days of malaise, headache, nausea, and vomiting, a 26-year-old man suddenly developed opsoclonus and stance and gait ataxia, without myoclonus. Having excluded a paraneoplastic etiology, we assumed that the disorder was probably related to a viral infection. Spontaneous resolution occurred in about two months. Opsoclonus became flutter dysmetria and then resolved. Saccadic eye movement recording disclosed the occurrence of hypermetria, increased velocity, and delayed latency, which also resolved. In this patient, the correspondence between clinical and ocular motor abnormality courses suggests a transient cerebellar dysfunction as the possible pathophysiologic mechanism for opsoclonus.
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PMID:Opsoclonus in a patient with cerebellar dysfunction. 1060 72

A 19-month-old boy presented with acute-onset cerebellar ataxia following immunisation. Ataxia was prolonged with a fluctuating course over a period of two years. Opsoclonus was never observed. Extensive diagnostic studies were negative during this time. Finally, a neuroblastoma was discovered. Ataxia disappeared completely during polychemotherapy. This report suggests that occult neuroblastoma must be considered in any child with unexplained prolonged cerebellar ataxia. Specific investigations are recommended for such cases.
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PMID:Late detection of neuroblastoma in a patient with prolonged cerebellar ataxia without opsoclonus. 1141 40

Opsoclonus is a rare but distinctive disorder of ocular motility, characterized by irregular, continual and conjugated chaotic saccades of the eyes. It is increased with eye closure and fixation, and it persists during sleep. Opsoclonus appears more commonly in children and in half of such cases in association with neuroblastoma. In adults the most frequent causes are idiopathic (50%) and paraneoplastic (20%). Among the second group, different types of tumors involving a wide variety of organs have been reported. Opsoclonus when accompanied by other symptoms of central nervous system involvement (head, appendicular myoclonus and truncal ataxia) constitutes a clinical picture, known as opsoclonus-myoclonus syndrome. We report the case of a young patient affected by renal cell carcinoma associated with opsoclonus-myoclonus syndrome which did not respond to medical therapy but dramatically improved after cancer ablation.
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PMID:Opsoclonus-myoclonus syndrome as a paraneoplastic manifestation of renal cell carcinoma. a case report and review of the literature. 1191 72

Opsoclonus-myoclonus-ataxia syndrome (OMA) in children is most commonly associated with occult neuroblastoma (NB). Although children with OMA and NB have decreased mortality, they suffer from increased neurologic morbidity. The pathogenesis of OMA in NB is not well understood, but current research and treatments support an immune-mediated process. The authors describe an unusual presentation of OMA occurring following 6 months of chemotherapy in a child with stage 4S NB who presented with partial Horner syndrome. Histopathologic examination of his primary cervical tumor showed NB maturation, which may have played a role in precipitating OMA syndrome. Further study of unusual cases of OMA in NB may provide better understanding of the syndrome and additional treatment options for these children.
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PMID:Late presentation of opsoclonus-myoclonus-ataxia syndrome in a child with stage 4S neuroblastoma. 1595 91

Opsoclonus-myoclonus-ataxia (OMA) secondary to Epstein-Barr virus (EBV) infection has only been described in three pediatric patients. Previous reports suggested that evidence for a recent EBV infection in the absence of an occult neoplasm would predict a favorable prognosis for OMA as well as no tumor development. We present the case of a 20-month-old child with OMA associated with a microbiologically documented acute EBV infection and an occult thoracic ganglioneuroblastoma diagnosed 5 months later.
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PMID:Epstein-Barr virus related opsoclonus-myoclonus-ataxia does not rule out the presence of occult neuroblastic tumors. 1620 May 67

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-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


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