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Miastenia gravis

Miastenia gravis. By : VIVI SOFIA, M.Si., Apt. Miastenia gravis adalah suatu penyakit autoimun dimana persambungan otot dan saraf ( neuromuscular junction ) berfungsi secara tidak normal dan menyebabkan kelemahan otot menahun. Clinical presentation. Causes Idiopathic Penicillamine

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Miastenia gravis

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  1. Miastenia gravis By : VIVI SOFIA, M.Si., Apt.

  2. Miastenia gravis adalahsuatupenyakitautoimundimanapersambunganototdansaraf( neuromuscular junction ) berfungsisecaratidak normal danmenyebabkankelemahanototmenahun.

  3. Clinical presentation • Causes • Idiopathic • Penicillamine • AChR antibodies are found in 90% of patients developing MG secondary to penicillamine exposure • Drugs

  4. Causes Drugs Antibiotics (Aminoglycosides, ciprofloxacin, ampicillin, erythromycin) B-blocker (propranolol) Lithium Magnesium Procainamide Verapamil Quinidine Chloroquine Prednisone Timolol Anticholinergics Clinical presentation

  5. Patofisiologi Sistemkekebalan yang membentukAntiboditubuh (Ig G) menyerangreseptor Ach yang terdapatpadasisiototdarineuromuscular junction akibatnyaterjadikekuranganrelatifdari Ach dipelatujungmotorisdariototlurik Kelemahanotot

  6. Patofisiologi (lanjutan) • melibatkan T-cell, B-cell dan tjd hiperplasia kelenjar timus (timoma) • ditemukannya antibodi anti Ach-R (anti asetilkolin reseptor).

  7. Pathophysiology • T-cell mediated immunity has some influence • Thymic hyperplasia and thymomas are recognized in myasthenic patients*

  8. Kelainan NMJ lainnya : • Sindrom Eaton-Lambert merupakanpenyakitnmjakibatpelepasan Ach yang tidakadekuat , biasanyaakibatefeksampingdarijeniskankertttterutamakankerparu-paru. • Botulisme akibatkeracunanmakanan yang tercemarbakteriClostridium botulinumygmelumpuhkanotot-ototdengancaramenghambatpelepasan Ach drsaraf. 3. Dosisantibiotikttt yang sangattinggi.

  9. EPIDEMIOLOGI • Lebih sering terjadi pada wanita ( F:M = 6 : 4 ) dan biasanya timbul pada usia 20-40 tahun. • Prevalensi di dunia 1 : 10.000

  10. GEJALA KLINIS • Kelemahanpadaototwajah • Kelemahanpadakelopakmata ( kelopakmatajauh ) • Kelemahanpadaototmata, sehing gaterjadipenglihatanganda. • Kelemahanpadalengandantung kai. 5. Kelelahanotot yang berlebihan setelahmelakukanolahraga. 6. Bisaterjadikesulitandalamberbi caradanmenelan. Sekitar 10% penderitamengalamikelemahanotot yang diperlukanuntukpernafasan.

  11. GEJALA KLINIS

  12. Diagnosa • Diagnosa ditegakkan berdasarkan gejala, yaitu jika seseorang mengalami kelemahan umum. • Tes edrofonium ( Pharmacological testing ) • Elektromiogram • Tes darah untuk mengetahui adanya antibodi dalam terhadap asetilkolin. • CT scan dada untuk menemukan adanya timoma.

  13. WorkupPharmacological testing Before After

  14. Treatment • AChE inhibitor • Pyridostigmine bromide (Mestinon) • Starts working in 30-60 minutes and lasts 3-6 hours • Individualize dose • Adult dose: • 60-960mg/d PO • 2mg IV/IM q2-3h • Caution • Check for cholinergic crisis • Others: Neostigmine Bromide

  15. Treatment • Immunomodulating therapies • Prednisone • Most commonly used corticosteroid in US • Significant improvement is often seen after a decreased antibody titer which is usually 1-4 months • No single dose regimen is accepted • Some start low and go high • Others start high dose to achieve a quicker response • Clearance may be decreased by estrogens or digoxin • Patients taking concurrent diuretics should be monitored for hypokalemia

  16. TreatmentBehavioral modifications • Diet • Patients may experience difficulty chewing and swallowing due to oropharyngeal weakness • If dysphagia develops, liquids should be thickened • Thickened liquids decrease risk for aspiration • Activity • Patients should be advised to be as active as possible but should rest frequently and avoid sustained activity • Educate patients about fluctuating nature of weakness and exercise induced fatigability

  17. Complications of MG • Respiratory failure • Dysphagia • Complications secondary to drug treatment • Long term steroid use • Osteoporosis, cataracts, hyperglycemia, HTN • Gastritis, peptic ulcer disease • Pneumocystis carinii

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