Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.1.8 (cholinesterase)
 12,691 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serum antibodies to muscle-specific receptor tyrosine kinase were detected in 12 of 32 patients with generalized seronegative MG. All were women, with onset between ages 21 and 59 years. Seven had prominent neck, shoulder, or respiratory muscle weakness and little or delayed ocular muscle involvement. The response to cholinesterase inhibitors was variable, and electromyographic findings suggested myopathy in several. None improved after thymectomy. All patients improved after plasma exchange, and most had a good response to selected immunotherapy. MuSK antibody status should help diagnose MG with atypical presentations and ensure appropriate patient treatment.
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PMID:Clinical aspects of MuSK antibody positive seronegative MG. 1282 44

Two boys, aged 2 and 11 years, presented with fever and muscle weakness that resulted in respiratory insufficiency. A physical examination and additional tests confirmed the diagnosis 'myasthenia'. Acetyl cholinesterase-inhibitor therapy had a favourable effect. Myasthenia is a diagnosis that should be considered for every child presenting with muscle weakness of unknown origin.
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PMID:[Two children seriously weakened by myasthenia]. 1510 20

We report a patient with polymyositis (PM) associated with myasthenia gravis (MG). Both disorders had been controlled for around 15 years by oral prednisolone and a cholinesterase inhibitor following surgical removal of invasive thymoma and radiotherapy, but muscular weakness due to myalgia and an increase in serum levels of myogenic enzymes, mainly ascribable to the recurrence of PM, reappeared immediately after cessation of these drugs, which was done because the patient had multiple bone fractures and severe osteoporosis due to the long-term corticosteroid therapy. Oral tacrolimus was therefore tried, and produced an improvement in muscular symptoms in association with normalization of myogenic enzymes. PM associated with MG as in this patient might be the best indication for tacrolimus, considering its efficacy in MG, but this drug should also be actively considered as a therapeutic option in refractory cases of PM alone, particularly when either corticosteroids or other immunosuppressive agents are not usable.
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PMID:Efficacy of tacrolimus in treatment of polymyositis associated with myasthenia gravis. 1516 59

Although once a severe and often fatal illness, myasthenia gravis can now be well managed with several relatively safe and effective therapies. Management involves a graded approach, beginning with cholinesterase inhibitors for mild symptoms and advancing to immunomodulating medications for more severe weakness. There are several immunomodulating agents from which to choose; selection is based largely on time to clinical effect and adverse effects. This review will discuss the selection and use of therapies for patients with myasthenia gravis.
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PMID:Management of myasthenia gravis. 1522 91

Organophosphates are commonly used as pesticides around the world. Exposures to organophosphates cause a significant number of poisonings and deaths each year. Organophosphates bind and inhibit cholinesterase enzymes. Acute toxicity manifests as a cholinergic crisis with excessive glandular secretions, altered mental status, and weakness. Several delayed syndromes have also been associated with organophosphate exposure, including a myasthenic-like syndrome, peripheral neuropathies, neuropsychiatric abnormalities, and extrapyramidal disorders. Clinical features and management of organophosphate poisoning is reviewed with emphasis on those affecting the central and peripheral nervous system.
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PMID:Organophosphate poisoning. 1525 17

To clarify the current status of treatments and outcomes of patients with myasthenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mean age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999-2000. Thymectomy was performed in 319 (68%) of the patients. Patients who received thymectomy were younger (p = 0.01) and had more severe disabilities (p < 0.01) than patients without thymectomy. Irrespective of receiving thymectomy, most of the patients were administered corticosteroids (64%), other immunosuppressive agents (10%), or cholinesterase inhibitors (86%). Of 395 patients followed up for more than 12 months after treatment (mean 8.0 years), 30% (34% of thymectomized and 21% of non-thymectomized patients) were in remission (no symptoms with/without medication), 34% had only ocular symptoms, and the remaining 35% still had weakness of bulbar or limb muscles at the end of follow-up. The prognosis of MG in Japan was generally favorable, but despite the frequent use of thymectomy and immunosuppressive treatments, approximately one-third of patients still had generalized weakness. More effective or intensive treatments are required to improve the prognosis.
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PMID:Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999-2000. 1545 Jul 70

Treatment for myasthenia gravis should be individualized to each patient based on the clinical characteristics of myasthenia including the distribution, duration, and severity of weakness and resulting functional impairment; the risks for treatment complications related to age, gender, and medical comorbidities; and the presence of thymoma. Acetylcholinesterase inhibitors provide temporary, symptomatic treatment for all forms of myasthenia gravis. Immune modulators address the underlying autoimmune process in myasthenia gravis, but are associated with potential complications and side effects. Most patients with generalized myasthenia who have significant weakness beyond the ocular muscles and who remain symptomatic, despite treatment with cholinesterase inhibitors, are candidates for immune modulation. Although corticosteroids are effective for long-term immune modulation in myasthenia gravis, several more contemporary immunomodulators including azathioprine, cyclosporine, and mycophenolate mofetil have shown efficacy in myasthenia gravis and are used increasingly as first-line treatments and as steroid-sparing agents. Plasma exchange is used to achieve rapid improvement in patients with myasthenic crisis or exacerbation, to improve strength before a surgical procedure or thymectomy, and to minimize steroid-induced exacerbation in patients with oropharyngeal or respiratory muscle weakness. Intravenous immunoglobulin represents an alternative to plasma exchange in patients requiring relatively rapid short-term improvement in the setting of poor venous access. Because of a lack of controlled trials, the role of thymectomy in nonthymomatous myasthenia gravis is unclear, although evidence suggests that thymectomy increases the probability for myasthenic remission or improvement.
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PMID:Autoimmune Myasthenia Gravis: Recommendations for Treatment and Immunologic Modulation. 1561 Jul 2

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of neuromuscular and autonomic transmission in which IgG autoantibodies lead to presynaptic voltage-gated calcium channel (VGCC) loss, or as a paraneoplastic disorder in association with small cell lung carcinoma (SCLC). Recent results strongly suggest that the antibodies to P/Q-type VGCC are the principal pathogenic factors in LEMS. Here, we present diagnosis and treatment of LEMS patients. Proximal weakness, depressed tendon reflexes, autonomic symptoms, and electrical posttetanic potentiation together are essential to accurately diagnose LEMS. The diagnosis is established immunologically by the presence of anti-P/Q-type VGCC antibodies, detected using the (125)I-omega-conotoxin MVIIC radioimmunoassay, which will be present in 85% of LEMS patients. The drug 3,4-diaminopyridine with anti-cholinesterase inhibitor is most effective in LEMS patients with or without SCLC. In LEMS with SCLC, specific tumor therapy will often improve the neurological disorder. In some cases plasmapheresis or intravenous immunoglobulin may be indicated.
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PMID:[Lambert-Eaton myasthenic syndrome: diagnosis and treatment]. 1577 66

Myasthenia gravis (MS) is an immune-mediated disorder characterized by fluctuating weakness and fatigue of voluntary muscles. The muscular disorder is generalized in 85% and confined to extraocular muscles in 15% of patients. Pathophysiology of MG involves generation of antiacetylcholine receptor antibodies, which leads to a reduction of the number of acetylcholine receptors at the muscular motor endplate. This in turn results in fewer acetylcholine receptors available for stimulation, lower amplitude stimulations, less muscle fiber activation, and the eventual development of weakness in the affected muscles. The diagnostic workup for MS consists of administration of anticholinesterase agents (Tensilon test), repetitive nerve stimulation, Ach-R antibody assay, and single-fiber electromyography. Management of patients with MG includes cholinesterase inhibitors, corticosteroids, thymectomy, immunosuppressants, plasma exchange, and IVIg.
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PMID:Management of myasthenia gravis. 1589 Dec 71

Myasthenia Gravis is an acquired autoimmune disorder caused by a neuromuscular transmission defect which is clinically characterized by fluctuating weakness of voluntary muscles and fatigability. It can be diagnosed by clinical features, clinical, pharmacological and electrophysiological tests and serological evaluation. Treatment modalities include symptomatic treatment in the form of cholinesterase inhibitors and plasmapheresis and immunotherapy in the form of immunosuppressant medications, immunomodulating therapy and thymectomy. No single regimen is appropriate for all patients and up to now no mode of therapy has been proven to be clearly superior. The response to any form of treatment is difficult to assess because the severity of symptoms fluctuate. We retrospectively analyzed the clinical records of 33 myasthenia gravis patients which were managed at our clinic between 1995-2003. All patients were treated with anticholinesterase medications sometime during their treatment. Most patients recieved immunosupressant and/or immunomodulator therapy. Patients were referred for thymectomy when indicated. We evaluated the outcome with different treatment modalities, focusing on the role of thymectomy. We also investigated the possible correlations between clinicopathological features and clinical outcome. We conclude that as for the medical treatment of myasthenia gravis azathioprine plus steroid improves the outcome; and for the surgical treatment, early thymectomy should be performed in all generalize myasthenia patients.
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PMID:Myasthenia gravis: how to treat? 1593 70


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