Lambert-Eaton myasthenic syndrome - Treatment

Treatment is individualized depending on the severity slabicunii, underlying disease, life expectancy and response to previous treatment. When confirming the diagnosis of Lambert-Eaton syndrome will look extensively associated neoplasia in the chest CT scan, bronchoscopy, radiography and PET. Initial treatment is aimed at eliminating cancer often fails because the weakness of effective therapy of cancer. Some patients may not even be a need for further treatment.If no tumor is found will regularly seek occult neoplasia. The frequency of these evaluations is determined by the patient's cancer risk. Patients under age 50 without smoking history of long-term reduced risk of associated neoplasia, especially if there is evidence of associated autoimmune diseases. Extensive surveillance for cancer in these patients may not be necessary.Syndrome in patients with cancer is usually not the main therapeutic care. Immunotherapy of cancer syndrome without effective treatment produces no improvement of weakness.Immunosuppressants used may promote tumor growth.
Pharmacological Therapy:Initial Pharmacotherapy Eaton-Lambert syndrome is done with agents that increase acetylcholine transmission along the neuromuscular junction, either by increasing or decreasing its release acetilcoliesterazelor action. Treatment also reduces cancer associated weakness and other symptoms.If these treatments are not effective and the patient has relatively mild weakness will determine whether aggressive immunotherapy is justified. When that is guaranteed to initially use intravenous immunoglobulin to induce a rapid improvement in transient.Imunosupresantele be added to a sustained improvement.
Neuromuscular Agents:These agents produce symptomatic improvement of the weakness, or both autonomous symptoms in patients with Lambert-Eaton syndrome. Is an inhibitor pyridostigmine acetilcoliesterazei and does not produce dramatic improvement but without weakness or dry mouth improvement in some patients.The preferred agent to be administered for several days before the onset of response.
Immunosuppressants:Prednisone and azathioprine, the most commonly used immunosuppressive agents can be administered alone or in combination. Cyclosporine immunosuppression may help patients candidates but do not respond well or can not take azathioprine.The improvement is seen in one month two months after starting cyclosporine, and the maximum response at 3-4 months.Plasmapheresis:Plasmapheresis produces improvement in many patients. The improvement is temporary and if the patient is receiving immunosuppressive therapy. Response to plasmapheresis in patients with Lambert-Eaton syndrome is often gradually towards those with myasthenia gravis. Maximum response may take several weeks. Repeated sessions of plasmapheresis are needed to keep improving. Plasmapheresis can be performed 4-6 times in 7-10 days as described in standard protocols. Potential complications include instability of autonomous, hypercalcemia, and bleeding due to depletion of clotting factors.
Human immunoglobulin therapy:Immune globulin administered intravenously at 2-5 days 2g/kg read also cause significant temporary clinical improvement in many patients. Frequency response improvement after repeated administration has not been determined.
Prognosis:
The prognosis is determined by the presence and type of cancer, the presence and severity of autoimmune disease and the severity and distribution associated with weakness. Patients with rapid and progressive symptoms are more severe disease. Evolution of patients without cancer is better compared to that of patients with cancer. When the syndrome was symptomatic peste2 years and has not shown any cancer is probably caused by an autoimmune process. At this point the prognosis is determined by the severity of dysfunction and the presence and severity of other autoimmune diseases.Without treatment weakness or dysfunction usually does not vary.Exceptions are periods of exacerbation or intercurrent diseases induced by drugs that affect neuromuscular transmission.
Drugs that exacerbate weakness in the Lambert-Eaton myasthenic syndrome:
Drugs that compromise neuromuscular transmission exacerbates weakness in syndrome. Competitive neuromuscular blocking agents such as tubocurarine and pancuronium have an exaggerated and prolonged effect. Initial signs of the syndrome include prolonged weakness or apnea after administration of agents during anesthesia.Some antibiotics, especially aminoglycosides, erythromycin fluoroquinolonele and neuromuscular blocking effects were significant. Some antiarrhythmics (procainamide, quinidine, quinine) and beta-adrenergic blocking agents worsen myasthenic weakness.Exacerbations of myasthenia after administration of magnesium and other agents including intravenous iodine contrast agents has been reported in isolated cases. If it is not absolutely necessary, avoid taking medications known to affect neuromuscular transmission.Muscle weakness is compounded when the ambient temperature increases or when the patient is feverish. Patients should avoid hot baths and showers.