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-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.
Pediatr Blood Cancer 2006 Dec
PMID:Epstein-Barr virus related opsoclonus-myoclonus-ataxia does not rule out the presence of occult neuroblastic tumors. 1620 May 67

Synaptic plasticity, the cellular correlate for learning and memory, involves signaling cascades in the dendritic spine. Extensive studies have shown that long-term potentiation (LTP) of the excitatory postsynaptic current (EPSC) through glutamate receptors is induced by activation of N-methyl-D-asparate receptor (NMDA-R)--the coincidence detector--and Ca(2+)/calmodulin-dependent protein kinase II (CaMKII). Here we report that the same signaling pathway in the postsynaptic CA1 pyramidal neuron also causes LTP of the slow inhibitory postsynaptic current (sIPSC) mediated by metabotropic GABA(B) receptors (GABA(B)-Rs) and G protein-activated inwardly rectifying K(+) (GIRK) channels, both residing in dendritic spines as well as shafts. Indicative of intriguing differences in the regulatory mechanisms for excitatory and inhibitory synaptic plasticity, LTP of sIPSC but not EPSC was abolished in mice lacking Nova-2, a neuronal-specific RNA binding protein that is an autoimmune target in paraneoplastic opsoclonus myoclonus ataxia (POMA) patients with latent cancer, reduced inhibitory control of movements, and dementia.
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PMID:Common molecular pathways mediate long-term potentiation of synaptic excitation and slow synaptic inhibition. 1621 16

Paraneoplastic Neurologic Diseases (PND) are remote complications of certain neoplastic diseases characterized by distinct neurological deficits in the central as well as the peripheral nervous system. The neurological disorders are usually clinically evident before the cancer is identified. PND are rare syndromes believed to have an immunological link where antibodies or activated T cells are acting against self antigens shared by the tumor cells and neurons. Most of these onconeural antigens are located in the cytoplasm-nuclear compartment of the cell, whereas others are located at the membrane and act as receptors or ion channels. This short review discusses the autoimmune mechanisms involved in PND in general and in opsoclonus-myoclonus-ataxia in particular. The article attempts to clarify why in most PND the immune effector mechanisms against onconeural antigens detected in sera of patients may not be the direct cause of the neurological deficit. We elaborate on the difference between the immune response against cytoplasmic-nuclear antigens as opposed to membranal antigens with regard to the fact that: a) the nervous system is considered immune privileged; b) neither the tumors nor the neurons have an optimal expression of MHC class I antigens and onconeural epitopes on the membrane. The availability of onconeural antigens to the immune effectors mechanisms is probably the main reason why there are few animal models for PND.
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PMID:[Autoimmune mechanisms in paraneoplastic neurological diseases: the paradigm of opsoclonus myoclonus ataxia syndrome]. 1664 34

Opsoclonus-myoclonus (OM) is a paraneoplastic syndrome of probable autoimmune origin. Despite current therapies aimed at decreasing autoantibody formation, OM is difficult to control and may impact long-term neurologic outcome. We present a case of a 19-month-old patient who initially presented with OM, neuroblastoma and a constitutional cytogenetic abnormality t(5;12)(q11.2;q15). The patient's OM was recalcitrant to conventional therapies, but showed significant improvement following treatment with rituximab.
Pediatr Blood Cancer 2008 Feb
PMID:Response to rituximab in a child with neuroblastoma and opsoclonus-myoclonus. 1665 44

Pain is not the most frequent symptom in cancer patients, but it is considered the most serious and threatening symptom. Opioid analgesics are the mainstay for analgesic treatment of cancer patients. Methadone is increasingly being considered as an alternative to morphine and seems to become relatively more potent with increasing exposure to other opioids. "Newer" side effects of opioids have been demonstrated, including cognitive impairment, generalized allodynia/hyperalgesia, myoclonus and hallucinations. When the pain relief effects of oral opioids have been exhausted, other pain management approaches, including spinal delivery of analgesics, may be effective alternatives.
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PMID:[Cancer-related pain]. 1676 93

Paraneoplastic neurological syndromes (PNS) are remote effects of cancer on the nervous system. An overview of the management of classical PNS, i.e. paraneoplastic limbic encephalitis, subacute sensory neuronopathy, paraneoplastic cerebellar degeneration, paraneoplastic opsoclonus-myoclonus, Lambert-Eaton myasthenic syndrome and paraneoplastic peripheral nerve hyperexcitability is given. Myasthenia gravis and paraproteinemic neuropathies are not included in this report. No evidence-based recommendations were possible, but good practice points were agreed by consensus. Urgent investigation is indicated, especially in central nervous system (CNS) syndromes, to allow tumour therapy to be started early and prevent progressive neuronal death and irreversible disability. Onconeural antibodies are of great importance in the investigation of PNS and can be used to focus tumour search. PDG-PET is useful if the initial radiological tumour screen is negative. Early detection and treatment of the tumour is the approach that seems to offer the greatest chance for PNS stabilization. Immune therapy usually has no or modest effect on the CNS syndromes, whereas such therapy is beneficial for PNS affecting the neuromuscular junction. Symptomatic therapy should be offered to all patients with PNS.
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PMID:Management of paraneoplastic neurological syndromes: report of an EFNS Task Force. 1683 98

Little information is available about the incidence, prevalence, or severity of morphine side effects during repeated individualized dosing for chronic cancer pain, although it has been widely used in this way for more than 30 years. The authors' aim was to describe the prevalence of symptoms possibly attributable to morphine side effects in a convenience sample of patients with pain due to advanced cancer. They used a prospective survey of inpatients and outpatients on regularly dosed morphine, with a questionnaire administered weekly for 4 weeks. Forty-two of 56 eligible patients completed at least the first questionnaire, with 30 completing all 4. Dry mouth was the most common symptom reported (point prevalence, 95%); this was often moderate to severe in intensity (57%) and was the most persistent symptom (period prevalence, 20%). Sedation and constipation were frequent (point prevalence, 88%) and was often moderate or severe at some point (55% and 62%, respectively) but had low period prevalence. Nausea was reported by less than half the patients. Myoclonus was common (point prevalence, 83%) but was usually mild and not persistent. Total daily morphine dose had little impact on side-effect patterns. Constipation, dysphoria, myoclonus, nausea, and sedation were more likely to be severe following dose increases. In conclusion, although constipation, nausea, and sedation are well described as side effects of morphine administration, others such as dry mouth and myoclonus appear to be underestimated. Validated patient-based measures of opioid side effects are needed.
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PMID:The adverse effects of morphine: a prospective survey of common symptoms during repeated dosing for chronic cancer pain. 1706 Feb 84

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.
Pediatr Blood Cancer 2008 Mar
PMID:Treatment of neuroblastoma-related opsoclonus-myoclonus-ataxia syndrome with high-dose dexamethasone pulses. 1722 43

The authors of this article present a case of a woman diagnosed with a vaginal melanoma who developed paraneoplastic syndrome (PNS) soon after diagnosis. A review of the literature regarding PNSs in gynecological malignancies is also described in this article. To our knowledge, this is the first reported case of paraneoplastic cerebellar degeneration with opsoclonus myoclonus secondary to a vaginal melanoma. In addition, our patient had an unusually acute progression to pancerebellar symptoms over the course of 3 weeks. Her paraneoplastic symptoms improved significantly after partial resection of the melanoma.
Int J Gynecol Cancer
PMID:Paraneoplastic syndrome in vaginal melanoma: a case report and review of the literature. 1730 66

Paraneoplastic neurological syndromes (PNS) can be defined as remote effects of cancer that are not caused by the tumor and its metastasis, or by infection, ischemia or metabolic disruptions. PNS are rare, affecting less than 1/10,000 patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small cell lung cancer. PNS can affect any part of the central and peripheral nervous system, the neuromuscular junction, and muscle. They can be isolated or occur in association. In most patients, the neurological disorder develops before the cancer becomes clinically overt and the patient is referred to the neurologist who has the charge of identifying a neurological disorder as paraneoplastic. PNS are usually severely disabling. The most common PNS are Lambert-Eaton myasthenic syndrome (LEMS), subacute cerebellar ataxia, limbic encephalitis (LE), opsoclonus-myoclonus (OM), retinopathies (cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), Stiff-Person syndrome (SPS), chronic gastrointestinal pseudoobstruction (CGP), sensory neuronopathy (SSN), encephalomyelitis (EM) and dermatomyositis. PNS are caused by autoimmune processes triggered by the cancer and directed against antigens common to both the cancer and the nervous system, designated as onconeural antigens. Due to their high specificity (> 90%), the best way to diagnose a neurological disorder as paraneoplastic is to identify one of the well-characterized anti-onconeural protein antibodies in the patient's serum. In addition, as these antibodies are associated with a restricted range of cancers, they can guide the search for the underlying tumor at a stage when it is frequently not clinically overt. This is a critical point as, to date, the best way to stabilize PNS is to treat the cancer as soon as possible. Unfortunately, about one-third of patients do not have detectable antibodies and 5% to 10% have an atypical antibody that is not well-characterized. As PNS are believed to be immune-mediated, suppression of the immune response represents another treatment approach.
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PMID:Paraneoplastic neurological syndromes. 1748 Feb 25


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