kkcase divisi Myastjjjjhenia Gravis.pptx

case divisi

1. Case Report : Myasthenia Gravis Ashifa Maulidya S Supervisor : dr. Andika Okparasta, Sp. S (K)

2. Patient Identity Name :Tn. F , Male Age : 32 Yo Marital status : Married Address : Palembang MRS : 08 November 2023 Keluhan Utama : Pasien datang dengan keluhan utama sesak nafas yang mengganggu aktivitas

3. Timeline Sesak (+) terutama di sore hari Batuk (+) Demam (-) Sulit menelan (+) sedikit -sedikit Sesak (+) Sulit menelan (+) Suara parau (+) Kelopak mata jatuh (+) Sesak memberat (+) kelemahan keempat ekstremitas Pasien dibawa ke RS 5 days Riwayat penyakit sebelumnya : 2018 , terdiagnosis MG --> tidak rutin berobat R/ Thymectomy pada tahun 2018 7 days 2 Minggu SMRS 1 minggu SMRS 1 hari SMRS

4. Physical Examination Sens : E 4 M 6 V 5 TD : 1 1- /70 mmHg HR : 70 x/m RR : 20 x/m T : 37.5 °c SpO2 : 98% Status Neurologis : N.III : pupil bulat, isokor N.III,IV,VI : kedudukan bola mata di tengah N.VII : plicanasolabialis simetris , sudut mulut simetris N.XII: deviasi lidah (-) , disartria (-)

5. Physical Examination Lka/ Lki/ Tka/ Tki Gerakan K/K/K/K Kekuatan 4/4/4/4 Tonus ↓ / ↓ / ↓ / ↓ Klonus - - R. Fisiologis ↓ / ↓ / ↓ / ↓ R. Patologis - / - / - / -

6. Laboratory Results ( 08 /1 1 /23) Hb : 11.8 WBC : 19.840 * Ht : 43 % RBC : 4.650.000 PLT : 636.000 Ca : 8.6 Na : 145 K : 5.0 Cl : 110 Chest X ray ( 08 /1 1 /23)

7. Diagnosis DK Tetraparese tipe flaccid Dyspnea Dysphagia Dysphonia Ptosis ODS DT NMJ DE Myasthenia Gravis Eksaserbasi akut MGFA IIIB Treatment Non Farmakologis O bservasi TTV dan Oksigenasi Adekuat RB PDL divisi Pulmo Farmakologis IVFD NS 0,9% gtt xx Inj. Methylprednisolon 500 mg / 24 jam (drip) Mestinon 3x60 mg PO Neurodex 1x tab Inj. Ceftriazone 2x1 gram iv N Acetyl sistein 3x200 mg PO

8. Follow Up 30 Nov 01 Dec Pulang perbaikan 10 Nov Sesak perburukan Krisis Myasthenia Intubasi dan pindah ICU Inj. Methylprednisolon 1x1 gr iv (tap off per 3 hari) Mestinon stop 15 Nov Sesak (-) Kelemahan ekstremitas (-) Dysphagia (-) Ptosis (-) Tx: - Mestinon 4x60 mg - Imuran 1x25 mg PO - Metilprednisolon 3x16 mg PO PLASMAPHARESIS 5X

9. Literature review

10. Introduction Myasthenia gravis (MG) is a relatively rare acquired, autoimmune disorder caused by an antibody-mediated blockade of neuromuscular transmission resulting in skeletal muscle weakness and rapid muscle fatigue . A utoantibodies form against the nicotinic acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles

11. Epidemiology 5 to 30 cases per million person-years Prevalence rate to be between 10 to 20 cases per 100,000 population Women > Men MG that start before the age of 18 years is almost 10% of all cases of MG Thymus abnormalities (10% patients) and thymic hyperplasia (30%) Ab against MuSK sarcolemmal protein (7%) and nicotinic AChR (85%)

12. In MG patients there is a decrease in the number or activity of ACh-R so decrease in the EPP, hence this potential may be sufficient at rest but with activities, there is not sufficient EPP to trigger an action potential.

13. Classification Depending on the type of clinical features and the type of antibodies involved, MG can be classified into various subgroups: Early-onset MG: Age at onset less than 50 years with thymic hyperplasia Late-onset MG: Age at onset greater than 50 years with thymic atrophy Thymoma-associated MG MG with anti-MuSK antibodies Ocular MG: Symptoms only from periocular muscles MG with no detectable AChR and MuSK antibodies

14. Klasifikasi Myasthenia Gravis Foundation of America (MGFA) Kelas I  : Adanya kelemahan otot-otot okular , kelemahan pada saat menutup mata , dan kekuatan otot-otot lain normal. Kelas II  : Terdapat kelemahan otot okular yang semakin parah , serta adanya kelemahan ringan pada otot-otot lain selain otot okular . Kelas IIa   : Mempengaruhi otot-otot aksial , anggota tubuh , atau keduanya . Juga terdapat kelemahan otot-otot orofaringeal yang ringan . Kelas IIb  : Mempengaruhi otot-otot orofaringeal , otot pernapasan atau keduanya . Kelemahan pada otot-otot anggota tubuh dan otot-otot aksial lebih ringan dibandingkan klas IIa . Kelas III   : Terdapat kelemahan yang berat pada otot-otot okular . Sedangkan otot-otot lain selain otot-otot ocular mengalami kelemahan tingkat sedang .

15. Kelas IIIa  : Mempengaruhi otot-otot anggota tubuh , otot-otot aksial , atau keduanya secara predominan . Terdapat kelemahan otot orofaringeal yang ringan . Kelas IIIb : Mempengaruhi otot orofaringeal , otot-otot pernapasan , atau keduanya secara predominan . Terdapat kelemahan otot-otot anggota tubuh , otot-otot aksial , atau keduanya dalam derajat ringan . Kelas IV  : Otot-otot lain selain otot-otot okular mengalami kelemahan dalam derajat yang berat , sedangkan otot-otot okular mengalami kelemahan dalam berbagai derajat . Kelas IVa : Secara predominan mempengaruhi otot-otot anggota tubuh dan atau otot-otot aksial . Otot orofaringeal mengalami kelemahan dalam derajat ringan . Kelas IVb : Mempengaruhi otot orofaringeal , otot-otot pernapasan atau keduanya secara predominan . Selain itu juga terdapat kelemahan pada otot-otot anggota tubuh , otot-otot aksial , atau keduanya dengan derajat ringan . Penderita menggunakan   feeding tube   tanpa dilakukan intubasi . Kelas V   : Penderita terintubasi , dengan atau tanpa ventilasi mekanik .

16. Clinical Manifestations The disease remains exclusively ocular in 10 - 40% of patients eye muscle weakness assymetrical and variable Bulbar muscle weakness - trouble whistling / smiling Weakness tends to spread from the ocular to facial to bulbar muscles and then to truncal and limb muscles. Limb weakness may be more severe proximally than distally Weakness is typically least severe in the morning and worsens as the day progresses . Weakness is increased by exertion and alleviated by rest. Weakness progresses from mild to more severe over weeks or months, with exacerbations and remissions.

17. Factors may trigger or worsen exacerbations Warm weather Surgery Immunization Emotional stress Menstruation Intercurrent illness (eg, viral infection) Tapering of immunosuppression Pregnancy and postpartum period Worsening of chronic medical illnesses (cardiac, renal, autoimmune, etc.) Medication (eg, aminoglycosides, ciprofloxacin, telithromycin. clindamycin, phenothiazines, chlorpromazine, diazepam, halothane, ketamine, lidocaine, procain, non-depolarizing neuromuscular blocking agents, chloroquine, procaine, lithium, phenytoin, beta-blockers, procainamide, calcium channel blockers, d-penicillamine, high-dose prednisone, magneisum).

18. Diagnostic procedures Immunological test Antibodies against acetylcholine receptors (Anti- AchR) is highly specific for MG, Anti- AchR not used to predict the severity of the disease, negative test results (Seronegativity) may occur if the test is done early or if it is immunosuppression condition . It has a sensitivity of approximately 85% for gMG and 50% for oMG. Tensilon (Edrophonium Chloride) Test Administered IV while the patient is observed for any objective improvement in muscle strength especially improvement in ptosis, while asymmetrical improvement in eyelid muscles is considered a positive result for the test. During this test, the patients must be monitored for cardiac and blood pressure - sensitivity of 71.5%–95% for the diagnosis of MG

19. Diagnostic procedures Ice pack test ice-pack placed over the eye for 2-5 minutes. Then, an assessment for any improvement in ptosis is done. ENMG Repetitive nerve stimulation (RNS) , introduced by Harvey and Masland (1941), has become the most routinely used test for the diagnosis of myasthenia gravis (MG). This is done by stimulating the nerve at 2-3Hz. Repeated nerve stimulation depletes the ACh in the NMJ, and produces a low excitatory postsynaptic potential (EPSP). A 10% or more decrease in the EPSP between the first and fifth stimulus is diagnostic of MG. sensitivity of this test is approximately 85% for gMG and 50% for oMG CT Scan / MRI for thymoma investigations Bedside examination Counting test, wartenberg test, diplopia stress test

20. Management strategies in MG are based on four principles: Symptomatic Treatment: Acetylcholinesterase inhibitors Pyridostigmine bromide is preferred over neostigmine because of its longer duration of action. Immunosuppresive : These are indicated in patients who remain symptomatic even after pyridostigmine treatment. 1st line agent : Glucocorticoids (prednisone, prednisolone, and methylprednisolone) and azathioprine 2nd -line agents : cyclosporine, methotrexate, mycophenolate, cyclophosphamide, and tacrolimus. Recently, various monoclonal antibodies, including rituximab and eculizumab, have been used to treat drug-resistant MG, but data from clinical trials about their efficacy is yet to be documented

21. Management strategies in MG are based on four principles: Intravenous immunoglobulins (IVIG) / Plasmapheresis This is recommended during the perioperative period to stabilize a patient before a procedure. It is also the treatment of choice for the myasthenic crisis due to its rapid onset of action and used in cases that are resistant to immunosuppressive drugs. Thymectomy: indicated for the following - Any subtypes of MG with evidence of thymoma. - Non-thymomatous n-AChR MG, especially in patients aged 15 to 50 years, performed 1-2 years of disease onset.[14] - Seronegative non-thymomatous MG.

22. Tatalaksana

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