Referat

Miastenia Gravis

Disusun Oleh: Agrita Eka Wulandari 0608120161 Diana Lestari 0608120093 Rosi Amalia 0708112073

Pembimbing : dr. Sutantri Edi Prabowo, SpAn dr. Soni, SpAn dr. Dino Irawan, SpAn

KEPANITERAAN KLINIK SENIOR BAGIAN ANESTESIOLOGI DAN REANIMASI FAKULTAS KEDOKTERAN UNIVERSITAS RIAU PEKANBARU 2011

KATA PENGANTAR Puji syukur kami panjatkan kehadirat Tuhan Yang Maha Esa, karena atas berkat dan rahmat-Nya, kami dapat menyelesaikan penyusunan referat yang berjudul MYASTHENIA GRAVIS. Referat ini kami susun untuk menambah ilmu pengetahuan yang kami miliki dan untuk melengkapi tugas di Kepaniteraan Klinik Anestesiologi Fakultas Kedokteran Universitas Riau di Rumah Sakit Umum Arifin Achmad Pekanbaru. Kami mengucapkan banyak terimakasih kepada semua pihak yang telah membantu, baik secara langsung maupun tidak langsung dalam menyusun referat ini. Kami menyadari masih banyak kekurangan baik pada isi maupun format referat ini, oleh karena itu, Kamimenerima segala kritik dan saran yang membangun demi kesempuranaan referat ini.

Pekanbar 6 September 2011

DAFTAR ISI

LEMBAR PENGESAHAN.. i KATA PENGANTAR. ii DAFTAR ISI iii BAB I : PENDAHULUAN. 1 BAB II : TINJAUAN PUSTAKA.. 1 II.1 Definisi..3 II.2 Prevalensi 3 II.3 Faktor resiko 3 II.4 Patofisiologi. .3 II.5 Manifestasi Klinis... 10 II.6 Pemeriksaan Penunjang 13 II.7 Diagnosis..15 II.8 Diagnosis Banding.. 17 II.9 Penatalaksanaan. 17 II.10 Komplikasi 20 II.11 Prognosis20 BAB III : KESIMPULAN.20 DAFTAR PUSTAKA........21

DAFTAR PUSTAKA 1. 2. 3. 4. 5. 6. 7. 8. Engel, A. G. MD. Myasthenia Gravis and Myasthenic Syndromes. Ann Neurol 16: Page: 519-534. 1984. Lewis, R.A, Selwa J.F, Lisak, R.P. Myasthenia Gravis: Immunological Mechanisms and Immunotherapy. Ann Neurol. 37(S1):S51-S62. 1995. Ngoerah, I. G. N. G, Dasar-dasar Ilmu Penyakit Saraf. Airlanga University Press. Page: 301-305. 1991. Howard, J. F. Myasthenia Gravis, a Summary. Available at : http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis.htm. Accessed : March 22, 2008. 9. 10. 11. 12. Newton, E. Myasthenia Gravis. Available at : http://en.wikipedia.org/wiki/Myasthenia_gravis. accessed : March 22, 2008. Murray, R.K, Granner, D.K, Mayes, P.A. Biokimia Harper: Dasar Biokimia Beberapa Kelainan Neuropsikiatri. Edisi 24. EGC. Jakarta. Page: 816-835. 1999. Anonim, Myasthenia Gravis. Available at: http://www.myasthenia.org/docs/MGFA_Brochure_Ocular.pd. Accessed: March 22, 2008. Anonim, Thymectomy, Available at : http://www.myasthenia.org/amg_treatments.cfm. Accessed : March 22, 2008.

Tugas referat 1 myastenia gravis Myasthenic crisis an acute episode of muscular weakness a lack of acetylcholine, usually caused by insufficient medication, which results in an inability to speak, breathe, or swallow in patients with myasthenia gravis. triggered by illness, infection, surgery, emotional stress, or an overdose or insufficiency of anticholinesterase medication. observations Typical signs and symptoms include worsening diplopia or muscle weakness that can progress to respiratory distress accompanied by apnea, extreme fatigue, increased muscular weakness, dysphagia, dysarthria, and fever. The patient may be anxious, restless, irritable, and unable to move the jaws or to raise one or both eyelids. If the condition is caused by anticholinesterase toxicity, then anorexia, nausea, vomiting, abdominal cramps, diarrhea, excessive salivation, sweating, lacrimation, blurred vision, vertigo, and muscle cramps and spasms, as well as general weakness, dysarthria, and respiratory distress may occur. Interventions of myasthenic crisis Initial treatment is directed to maintaining airway patency. Oxygen with assisted or controlled ventilation is administered. The patient is placed in a bed in which the head is elevated 30 degrees.

 The withdrawal or reduction of anticholinergic drugs may be ordered, or they may be given to differentiate the kind of crisis. If the eyelids are affected, the eyes may be covered with a patch and soothing eyedrops may be administered. To enable the patient to communicate, the call bell and a pad and pencil are placed within reach. Nourishment is offered between meals, and a daily intake of up to 2000 mL of fluids is encouraged. Walking as tolerated and other activities are planned at the time of the maximum effect of medication. Active or passive range-of-motion exercises of all extremities are performed several times a day, but rest periods are maintained to prevent fatigue and relapse.

2.9 Penggunaan Obat-obatan Anestesi 2.9.1 Anestesi Umum Pasien dengan penyakit junctional yang mendasari seperti Miestenia Gravis (MG) yang dapat menyebabkan kelemahan berkepanjangan memiliki prosedur-prosedur tertentu dalam memberikan obat-obat anestesi untuk tujuan pembedahan. Terdapat banyak faktor yang

mempersulit melakukan tindakan anestesi pada pasien dengan MG. Anestesi umum dapat memperberat terjadinya penghambatan neuromuscular pada pasien dengan MG terutama pada penggunaan anestesi inhalasi yang memberi dampak langsung pada transmisi neuromuscular. Administrasi rutin inhalansia anestesi methoxyflurane dilaporkan untuk meng-unmask MG.82 ringan Dengan paparan berulang pasien dikembangkan kelelahan, kelemahan dan ptosis selama beberapa jam, yang membaik setelah CEI. Dalam studi eksperimental beberapa inhalasi anestesi muncul untuk mengubah pasca-junctional kepekaan terhadap AcH, mempengaruhi konduktansi ionik, dan menyebabkan pemendekan waktu diaktifkan AcH-saluran terbuka 2.9.2 Anestesi Lokal Pada orang normal lokal menggunakan anestesi adalah tidak mungkin untuk menyebabkan kelemahan neuromuskular yang signifikan. Intravena lidokain, prokain, dan anestesi lokal agen dapat mempotensiasi efek obat memblokir neuromuskuler. Tampaknya ada kedua efek presynaptic dan postsynaptic. Interferensi dengan propagasi dari potensial aksi saraf pada terminal saraf dan mengurangi AcH rilis dapat menjelaskan efek presynaptic. 84 anestesi lokal juga menyebabkan berkurangnya sensitifitas membran postjunctional untuk acetylcholine.85 Harvey diamati prokain untuk menginduksi myasthenic krisis akut pada 1939, namun penelitian selanjutnya memberikan bukti sebaliknya.

Neuromuscular Blocking Drugs Depolarizing and nondepolarizing neuromuscular blockers affect the muscle membrane potential. Neuromuscular blocking drugs may be modified by the degree of neuromuscular block, the associated disease state, acid base status of the patient, and associated electrolyte imbalance. In patients with MG and Lambert Eaton syndrome relatively small amounts of nondepolarizing agents can produce profound and prolonged NMJ blockade. Memblokir neuromuskular Obat Depolarizing dan neuromuskuler blocker nondepolarizing mempengaruhi potensial membran otot. Obat memblokir neuromuskuler dapat dimodifikasi dengan tingkat blok neuromuskuler, keadaan penyakit terkait, asam Status dasar pasien, dan ketidakseimbangan elektrolit terkait. Pada pasien dengan MG dan Lambert Eaton syndrome jumlah yang relatif kecil dari agen nondepolarizing dapat menghasilkan blokade NMJ mendalam dan berkepanjangan

Prolonged paralysis from neuromuscular blockers may occur on occasion in patients without NMJ disease. Factors which may influence the duration of neuromuscular blockade include dosage and duration of therapy, concurrent drug use (including muscle relaxants, magnesium, and cimetidine), severity of underlying disease, electrolyte abnormalities, and renal insufficiency (which may lead to high drug concentrations).88 Use of muscle relaxants for one week in a child resulted in a six week course of recovery.89 Similarly, long-term weakness from vecuronium use is reported to occur in adults.90,91 Paralysis lasted eight weeks after use of the neuromuscular blocker atracurium besilate.92 Patients with metabolic acidosis, high serum magnesium levels, renal failure, and high blood levels of 3-desacetyl-vecuronium appear more likely to experience prolonged paralysis.93 Prolonged neuromuscular blockade can be severe enough to produce neurogenic muscle atrophy.94 Corticosteroids may even potentiate the effects of muscle relaxants.95 Prolonged weakness in intensive care patients can result from a multitude of causes, some leading to peripheral neuropathy and myopathy as well as junctional disturbance as above. In many patients there are multiple concomitant factors leading to the paralytic picture. Depolarizing agents, including succinylcholine, should be used with caution in patients with known NMJ disease. The inhibition of hydrolysis by cholinesterase inhibitors will result in prolonged duration of action. Patients with MG are less sensitive to this drug than the nondepolarizing agents. Occasionally, patients with MG have their disease unmasked by the use of these drugs. Pharmacological effects of neuromuscular blockers are influenced by antibiotics, general anesthetics, local anesthetics, and antiarrhythmics which may complicate clinical weakness. Newer neuromuscular blocking agents having shorter duration of action may still aggravate MG and Lambert-Eaton syndrome weakness. Occasional reports of reduced plasma cholinesterase levels by plasma exchange or by genetic abnormalities have been associated with reports of prolonged apnea and muscle weakness in patients receiving depolarizing neuromuscular blocking drugs.