Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Opsoclonus-myoclonus is a pervasive neurological syndrome of children and adults. Although rare, it raises important clinical and neurobiological issues. This article provides an overview of the clinical and laboratory features, differential diagnosis, treatment, and outcome of opsoclonus-myoclonus. It pursues immunologic, genetic, electrophysiologic, neurochemical, and other clues to a pharmacologic model. Key questions include how and where the brain is injured, reversibility of the injury, possible targets for pharmacologic intervention, and which new studies are needed.
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PMID:The neurobiology of the opsoclonus-myoclonus syndrome. 139 42

We report 7 patients who developed acute co-occurrences of fragmentary generalized myoclonus and asterixis. All patients were elderly and had other chronic diseases. This condition appeared acutely, progressed over several hours and then disappeared in 2-3 days with diazepam administration. No sequelae were noted, although most cases developed recurrences. The myoclonus occurred spontaneously and was slightly enhanced by action. The myoclonus was widely distributed but predominated in the neck, shoulder girdle, and upper extremities. Opsoclonus was not noted. Clinically apparent myoclonus was not evoked by sensory stimuli. Asterixis was observed in the upper extremities in all cases. Asterixis-like movements of the protruded tongue were also observed. Neurological findings other than the myoclonus and asterixis were unremarkable. Neither metabolic nor organic abnormalities clearly responsible for this condition were identified. Cerebral potentials preceding the myoclonic jerks recorded in one case suggested that the myoclonus may have been a spontaneous cortical myoclonus. We named this condition a transient myoclonic state with asterixis (TMA). Awareness of this syndrome is clinically important because of its benign nature, although it can recur.
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PMID:Transient myoclonic state with asterixis in elderly patients: a new syndrome? 163 95

Opsoclonus-myoclonus (OM) is a neurological disorder usually occurring in infancy, clinically manifested by various involuntary movements. The pathogenesis of OM is unknown, but since the disease often is associated with viral infection or with neuroblastoma, an immunologic basis for OM has been postulated. We have studied two children with OM whose serum contained antibodies directed against the 210 kDa neurofilament protein; these antibodies were not seen in the serum of 21 children with other neurological disorders. Neurofilament proteins, which are found only in neurons, may be of prime importance in neuronal function, especially during development of the nervous system. Our findings suggest that generation of antibodies to the neurofilament proteins can occur in patients with opsoclonus-myoclonus; the role of the anti-NF210K antibodies in the pathogenesis of OM, however, is uncertain.
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PMID:Anti-neurofilament protein antibodies in opsoclonus-myoclonus. 329 94

An increased compendium of drugs useful in ocular motor system dysfunction has expanded our capacity to treat selected ocular motility disorders. Adjunctive therapeutic modes (e.g., Fresnel prisms and orthoptic exercises) can also be beneficial. PAN and see-saw nystagmus can be treated with baclofen. Downbeat nystagmus may respond to clonazepam therapy, and prisms may help if the nystagmus can be modified with convergence. Congenital nystagmus may respond minimally to drugs (e.g., baclofen), but prisms or surgical procedures, or both, are still the primary treatment modalities. Innovar may be helpful in patients with severe, incapacitating vestibular disorders, and scopolamine alone or in combination with promethazine may be beneficial in patients with milder ambulatory acute peripheral vestibular disorders. Benign positional vertigo is best treated initially with positional exercises before drug therapy is instituted. Opsoclonus and ocular flutter have been treated successfully with corticosteroids, propranolol, and clonazepam, while microflutter, an extremely rare disorder, can resolve with baclofen. Although therapy with carbamazepine, 5-hydroxtryptophan, and scopolamine has been useful in selected patients with ocular palatal myoclonus, most do not respond to drug treatment. It is not usually necessary to treat voluntary nystagmus, but Fresnel prism lenses should be remembered in refractory patients. Potentially reversible and pseudointernuclear ophthalmoplegias also were discussed. Orthoptic exercises can be beneficial in posttraumatic internuclear ophthalmoplegia. Selected supranuclear palsies can be improved completely with the proper drug regimen. Lastly, superior oblique myokymia can be treated successfully with carbamazepine, with tight surveillance for possible adverse side effects. Descriptive phenomenology and pathophysiological localization must be correlated with brain stem neurochemistry and neuropharmacology to medically treat additional ocular motor system disorders.
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PMID:Medical treatment of nystagmus and ocular motor disorders. 349 76

Neuroblastoma is one of the commonest childhood malignancies. The most important prognostic factor is age at diagnosis; early diagnosis, when the tumor is still localized and surgically resectable, is second in importance. On retrospective review of children seen at the Hospital for Sick Children, ophthalmic involvement was seen in 80 of 405 (20%). The three major eye signs of neuroblastoma, proptosis, Horner's syndrome and opsoclonus, are closely related to the site, stage of tumor, and outcome of the patient. Proptosis or periorbital ecchymosis due to orbital metastases was present in 60 of 80 children (bilaterally in 33). The 3-year survival rate was 11.2%. In 53 of 60 cases with orbital metastases the neuroblastoma originated in the abdomen. Unilateral Horner's syndrome occurred in 14 children, as the presenting sign in 9, related to localized disease in 11 and in a favorable location (cervical or thoracic neuroblastoma) in 8. The 3-year survival rate was 78.6%. Opsoclonus-myoclonus was the presenting sign of occult, localized neuroblastoma in all 9 children in whom it occurred. The 3-year survival rate was 100%. For all presentations, girls had a significantly better survival rate than boys (48.7% vs. 22.4%). Children presenting with any of these ophthalmological signs should undergo thorough and repeated investigations searching for neuroblastoma.
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PMID:Ocular involvement in neuroblastoma: prognostic implications. 649 2

Opsoclonus-myoclonus is a rare clinical syndrome. We report three patients who died with a pure opsoclonus-myoclonus syndrome. The presentation, management and associations of this distinctive condition are discussed, and the results of detailed pathological examination described. In each case the only abnormality was perivascular collections of lymphocytes widely distributed in the brain. This supports an immune-mediated rather than structural aetiology.
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PMID:Opsoclonus-myoclonus syndrome: an autopsy study of three cases. 826 Oct 78

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

The different pathogenic and clinical aspects of the paraneoplastic ophthalmological syndromes are discussed by the author. Opsoclonus-myoclonus, retinal degeneration are largely reviewed. It is possible that new modern immunological methods to elucidate the role of antibodies in the genesis of paraneoplastic syndrome and the role of these antigens in the functioning of nervous system. Recognising a ophthalmological syndrome as paraneoplastic will prompt a search for an early detection of a cancer and a more prompt treatment.
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PMID:Paraneoplastic ophthalmological syndromes. 891 42

A case of Opsoclonus-Myoclonus occurring in a young man, in association with traditional herbal medicine consumption is presented. Clinical and laboratory investigations did not reveal any of the known aetiological associations of the Opsoclonus-Myoclonus syndrome, raising the possibility that the traditional herbal medicine may be aetiologically implicated. This report highlights the need for proper identification and documentation of the contents of common herbal remedies and their possible side effects amongst Africans.
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PMID:Opsoclonus-myoclonus associated with traditional medicine ingestion: case report. 964 Aug 38

Opsoclonus-myoclonus is a well-described paraneoplastic syndrome that most often occurs in association with small cell carcinoma of the lung and breast carcinoma. To the best of our knowledge, we describe the first reported case of paraneoplastic opsoclonus-myoclonus occurring is association with malignant melanoma. Antineuronal antibodies were not identified despite repeated testing. No therapeutic benefit was observed following corticosteroid administration.
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PMID:Paraneoplastic opsoclonus-myoclonus secondary to malignant melanoma. 1022 21


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