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)

Paraneoplastic cerebellar degeneration (PCD) is a rare complication of systemic cancer. PCD may present as a "pure", severe pan-cerebellar syndrome of subacute progression or be only one clinical feature in the setting of extensive CNS disease. The most characteristic form of "pure" PCD is associated with the presence of an anti-Purkinje cell antibody (AB), called anti-Yo, in patients with breast or ovarian cancer. The primary tumor is very often unknown when the cerebellar signs occur, and extensive investigations, including laparotomy or prolonged follow-up may be required to demonstrate its presence. More rarely, others AB than anti-Yo are discovered during PCD. Almost 50% of patients with "pure" PCD do not have circulating anti-neuronal AB. In the cases, the primary cancer is more often known and the clinical course of the cerebellar syndrome may be slower. Cerebellar degeneration may also occur during paraneoplastic encephalomyelitis. In this setting, the cerebellar signs which may be isolated at the onset, become associated with other signs of neuraxis involvement (limbic encephalitis, brainstem encephalitis, myelitis and particularly, subacute sensory neuronopathy) during the course of the disease. When a paraneoplastic encephalomyelitis is associated with a small cell lung cancer, an antineuronal AB called anti-Hu is frequently found. Finally PCD may be associated with the opsoclonus-myoclonus syndrome with the Lambert-Eaton syndrome.
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PMID:[Paraneoplastic cerebellar degeneration]. 786 50

The opsoclonus-myoclonus syndrome is a rare entity as a paraneoplastic disorder usually associated to neuroblastoma in children and breast cancer or oat-cell lung carcinoma in adults. The association of opsoclonus-myoclonus syndrome and ovarian carcinoma is very unusual, to our knowledge there is only two cases reported in the literature. In both of them the opsoclonus-myoclonus syndrome preceded the neoplasy, improving with its treatment. In our patient opsoclonus began after ovarian cancer diagnosis, after chemotherapy and radiotherapy, improving with corticoid and clonazepan therapy.
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PMID:[Opsoclonus-myoclonus syndrome in patient with ovarian cancer]. 1289 56

A 55-year-old woman with advanced ovarian cancer and severe pain developed hypoactive delirium after an increase in her opioid dosage. Myoclonus and delirium improved dramatically with the intravenous injection of the acetylcholinesterase inhibitor physostigmine, and this improvement was maintained during the administration of donepezil, an oral medication with similar pharmacodynamic properties. Evidence for a disorder of cholinergic neurotransmission in opioid-induced delirium is discussed, as is the rationale for treatment with acetylcholinesterase inhibitors and other cholinomimetic agents.
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PMID:Treatment of opioid-induced delirium with acetylcholinesterase inhibitors: a case report. 1503 35

Opsoclonus myoclonus with ataxia (OMA) is a rare neurological disorder. The syndrome is characterized by involuntary, conjugate, multidirectional eye movements accompanied by involuntary movements of limb or face, and sometimes ataxia, dysarthria, irritability, dementia, altered level of consciousness and even death. OMA is associated with various etiologies including infectious, toxic, drug-related, vascular and paraneoplastic conditions. Paraneoplastic opsoclonus myoclonus with ataxia (POMA) is more common in patients over 40 years of age and is usually associated with lung (especially small cell), breast and ovarian cancer but has also been reported with many other cancers. The syndrome is thought to be mediated by autoantibodies directed against onconeural antigens that are expressed by the tumor as well as by neurons. Studies from several laboratories were able to demonstrate a role for the cellular response in the pathogenesis of POMA. The results for treatment of this syndrome have been disappointing, although aggressive multimodality immunosuppressive treatments have been used. This is a case study of a patient with POMA who clearly demonstrates the difficulties in the diagnosis and treatment of this syndrome.
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PMID:[Opsoclonus myoclonus with ataxia]. 1584 53

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