{"id":222,"date":"2012-06-27T12:18:35","date_gmt":"2012-06-27T12:18:35","guid":{"rendered":"http:\/\/www.myotonia.com.hr\/en\/treatment\/"},"modified":"2012-06-27T12:35:30","modified_gmt":"2012-06-27T12:35:30","slug":"treatment","status":"publish","type":"page","link":"https:\/\/www.myotonia.com.hr\/?page_id=222","title":{"rendered":"Treatment"},"content":{"rendered":"<p style=\"text-align: justify;\">In patients with mild clinical symptoms, drug treatment is often unnecessary.<\/p>\n<p style=\"text-align: justify;\">Life style<\/p>\n<ul style=\"text-align: justify;\">\n<li>Relaxation techniques and stress avoiding (well known provocative factor) are beneficial<\/li>\n<li>Avoiding periods of longer rest or maintenance of same position helps with muscle stiffness control<\/li>\n<li>Sudden movements after prolonged rest should be avoided in order to prevent muscle injuries and falls<\/li>\n<li>Warm up phenomenon<\/li>\n<li>Exercise and stretching is recommended to improve flexibility, especially in Achilles tendons which frequently require surgical lengthening<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">\u00a0<strong>Drug treatment<\/strong><\/p>\n<p style=\"text-align: justify;\">\u00a0Mexiletine<\/p>\n<ul style=\"text-align: justify;\">\n<li>Mexiletine is most often the first choice for treatment of myotonia, based on its efficacy and mostly benign adverse effect profile. It is a antiarrhythmic agent, which acts on fast-acting voltage-gated sodium channels. Its half-life is 10 to 12 h.<\/li>\n<li>Standard dosage is 150 mg to 200 mg\u00a0 in adults and 1 to 8 mg\/kg per day given two to three times a day given with meal in order to prevent gastrointestinal side-effects.<\/li>\n<li>Treatment starts with lowe dose which increases over time. Patients are advised to stop tretament every few years to sure that treatment is still warranted<\/li>\n<li>Because rapid discontinuation can cause a rebound in myotonia and stiffness, the drug should be tapered in 50-mg increments every few days.<\/li>\n<li>Contraindications: patients with severe cardiac arrhythmias, cardiomyopathy, or coronary artery disease. It should also not be used in patients with an allergic response to lidocaine or lidocaine derivatives<\/li>\n<li>Side-effects: epigastric symptoms (20% of patients) that can be alleviated if the drug is taken with food, lightheadedness (10%,), skin rash (3%). Other side-effects are anxiety, headaches, and coordination difficulties.<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">\u00a0Tocainide<\/p>\n<ul style=\"text-align: justify;\">\n<li>Tocainide is a lidocaine derivative that acts on the sodium channel. It has been shown to be as effective as mexiletine in controlling myotonia. However, because it has a depressive effect on bone marrow, it is considered a second-line agent for the treatment of myotonia congenita. Its half-life is 15 h.<\/li>\n<li>Standard dosage is 200 mg twice a day. It can be increased up to 1,200 to 1,600 mg\/d divided in two or three doses<\/li>\n<li>Contraindications: patients with severe cardiac arrhythmias, cardiomyopathy, or coronary artery disease and patients allergic to lidocaine and its derivates. Tocainide should be avoided in any patient with a history of bone marrow suppression.<\/li>\n<li>Side effects: dizziness (11%), lymphadenopathy (3%), nausea, anxiety, and tremor.<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">\u00a0Carbamazepine<\/p>\n<ul style=\"text-align: justify;\">\n<li>Carbamazepine works by reducing post-tetanic potentiations and is thought to stabilize the muscle membrane. Its half-life is 12 to 17 h.<\/li>\n<li>Contraindications: hypersensitivity to tricyclic antidepressants or a history of recent use of monoamine oxidase inhibitors. It also should be avoided in patients with a history of increased intraocular pressure, liver and kidney disease<\/li>\n<li>Side effects : somnolence, changes in ECG, dizziness, blurred vision, lack of coordination, increased liver enzymes<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">Phenytoin<\/p>\n<ul style=\"text-align: justify;\">\n<li>Phenytoin works as voltage-dependent sodium and calcium channel modulator<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">Drugs to avoid<\/p>\n<ul style=\"text-align: justify;\">\n<li>Beta-agonists, beta-blockers, acetilcholiesterase inhibitors \u2013 worsen myotonia<\/li>\n<li>Colchicin- causes muscle weakness<\/li>\n<\/ul>\n<p style=\"text-align: justify;\">\u00a0Anesthesia in patients with myotonia<\/p>\n<ul>\n<li style=\"text-align: justify;\">Patients with congenital myotonia have an increased risk for development of malignant hyperthermia<\/li>\n<li style=\"text-align: justify;\">Suxamethonium, succinylcholine, and propofol can induce myotonia<\/li>\n<li style=\"text-align: justify;\">It should be taken into consideration that patients should be kept warm during surgery<\/li>\n<\/ul>\n","protected":false},"excerpt":{"rendered":"<p>In patients with mild clinical symptoms, drug treatment is often unnecessary. Life style Relaxation techniques and stress avoiding (well known provocative factor) are beneficial Avoiding periods of longer rest or maintenance of same position helps with muscle stiffness control Sudden movements after prolonged rest should be avoided in order to prevent muscle injuries and falls Warm up phenomenon Exercise and stretching is recommended to improve flexibility, especially in Achilles tendons which frequently require surgical lengthening [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"open","ping_status":"open","template":"","meta":{"footnotes":""},"class_list":["post-222","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/pages\/222"}],"collection":[{"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=222"}],"version-history":[{"count":3,"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/pages\/222\/revisions"}],"predecessor-version":[{"id":251,"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=\/wp\/v2\/pages\/222\/revisions\/251"}],"wp:attachment":[{"href":"https:\/\/www.myotonia.com.hr\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=222"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}