Motor Neuron Disease 22
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Transcript of Motor Neuron Disease 22
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NEUROMUSCULAR DISEASES AND NEUROPATHY
SURYADI
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HORNER’S SYNDROME
Penyebab :- Lesions sympathetic fibers
( hypothalamus to cervical cord, C8 – T2 roots to superior cervical ganglion, cavernous sinus)
Gejala :- Miosis- Ptosis(kelopak mata jatuhg’ bs terbuka)
- Anhidrosis hemifasialis ipsilateral- Hiperemia hemifasialis ipsilateral- Best seen in dark- Pupillary asymmetry
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CARPAL TUNNEL SYNDROME (CTS)
adalah problema jebakan saraf perifer paling umum yang mengenai tangan dan pergelangan tangan, mengenai 1% masarakat dan 80% mengenai wanita.
kompresi n. medianus di terowongan karpal. Tulang karpal membentuk lantai dan dinding terowongan karpal, dan atap dibentuk oleh ligamentum karpal transversum. 9 tendon fleksor dengan sarungnya menyertai n. medianus didalam terowongan karpal.
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• CTS terjadi dalam keadaan pengurangan ruangan di terowongan atau disebabkan peningkatan kerentanan saraf.
• disebabkan oleh tenosynovitis, RA(reumatoid-artritis), ganglia, osteofit, osteosis, anomali otot, atau tumor.
• Retensi cairan diperkirakan terjadi selama kehamilan, selama laktasi
• Kondisi peningkatan kerentanan saraf pada DM, hipothiroid,
• karena pekerjaan dengan gerakan repetitif tangan dan pergelangan tangan dan terlihat pada bermacam akitifitas dan okupasi.
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GEJALA
• Nyeri, rasa pedih atau rasa baal pada ibu jari, jari telunjuk, jari tengah atau jari manis, khususnya memburuk saat malam.
• Rasa pedih atau rasa baal di seluruh tangan.
• Nyeri yang menyebar dari tangan ke lengan sejauh bahu.
• Perasaan bengkak atau ketat pada tangan atau pergelangan tangan.
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Sindroma Terowongan Karpal
• N. Medianus terjebak dlm Terowongan Karpal• Kesemutan pada Jari I, II dan III• Pada Stadium lanjut : terjadi atropi otot Tenar• Diagnosis berdasarkan :
-Gejala klinis -Tes Phalen-Elektromiografi
• Stadium dini dgn istirahat & Fisioterapi• Pengobatan dgn suntikan Steroid lokal• Stadium lanjut : Operasi
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Kadangkala dapat juga terjadi :
• Satu atau beberapa gejala tersebut diatas memburuk pada malam hari atau ketika bangun tidur di pagi hari.
• Tangan atau lengan bawah terasa lemah di pagi hari.• Menjatuhkan benda lebih sering daripada biasanya.• Kesulitan menjepit atau menggenggam obyek.• Kesulitan melakukan tugas yang detail seperti menulis atu
mengikat tali sepatu.• Kesulitan melakukan tugas yang memerlukan kekuatan
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3 tes provokasi :
• tanda Tinel : parestesi dipicu dengan mengetuk
n. medianus di pergelangan tangan.
• manuver Phalen : menahan fleksi pergelangan
tangan menghasilkan parestesi dalam 1 menit.
• tekanan langsung terowongan karpal : tekanan
diatas terowongan karpal selama 30 detik.
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Pemeriksaan motorik :
– Lihat adanya atrofi eminensia thenar.
– Kelemahan bahkan atrofi m. abduktor pollicis brevis, m. opponens pollicis, mm. Lumbrikales I & II, m. fleksor pollicis brevis.
- Uji kekuatan abduksi dan oposisi ibu jari.
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PENCEGAHAN
• Hindari atau kurangi jumlah gerakan berulang pergelangan tangan sedapat mungkin.
• Gunakan peralatan dan alat yang dirancang secara tepat untuk mengurangi risiko cedera pergelangan tangan
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PENGELOLAAN• Wrist splint : plastik ringan posisi netral / Velcro
splints yang mengizinkan gerakan jari setengah bebas. 22% penderita bebas gejala sesudah 1 tahun jika diobati dengan splinting.
• Modifikasi aktifitas : mengurangi gerakan fleksi, ekstensi, rotasi pergelangan tangan, fleksi jari-jari dan menggenggam dengan kuat.
• Ultra Sound Diathermi• NSAID• Diuretik untuk penderita dengan pembengkakan
anggota gerak.
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TARSAL TUNNEL SYNDROME
Jebakan saraf di terowongan tarsal yg dibentuk oleh lig lasiniatum yg terbentang dr bag bawah blk maleolus medial tibiae dan tuber kalkanei
Gejala : rasa baal, spt kecabaian, dan sebagainya
Karena jebakan saraf diantara os metatarsale dapat menimbulkan pembengakakan
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NEUROPATI
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PENDAHULUAN
• Nyeri neuropatik banyak dijumpai
• Penyebabnya bermacam-macam
• Terapinya beraneka-ragam
• Mempunyai dampak sosio-ekonomi
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DEFINISI NYERI (IASP)
Pengalaman sensorik yang tidak
menyenangkan, berhubungan dengan
kerusakan jaringan yang aktual ataupun
potensial
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NEUROPATHY
Progressive degeneration of • anterior horn cells• corticospinal fibers, and• motor nuclei in the medulla
Various levels of the nervous system:
bulbar, cervical, and lumbar may be involved All level of the motor system are involved
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• Nyeri merupakan pengalaman psikik normal dari suatu penyakit, berhubungan dengan kerusakan jaringan atau kerusakan yang mengancam jaringan badan.
• melibatkan dua proses : 1) Proses perifer berhubungan dengan deteksi dan transmisi informasi yang berhubungan dengan kerusakan jaringan ; 2) Proses sentral yang menguasai respon psikik atau respon serebral terhadap informasi.
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Neuropati
• ialah keadaan dimana saraf tepi mengalami gangguan fungsi akibat kerusakan seluler ataupun molekuler, dan dapat disebabkan oleh berbagai macam etiologi seperti : trauma, entrapment (terjepit / terjebak), penyakit metabolik, penyakit defisiensi , keracunan (zat kimia toksik, logam berat), gangguan imunologis, bahkan etiologi yang sifatnya genetik.
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• mengenai banyak saraf tepi dan distribusinya umumnya bilateral simetris
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Kategori Klinis Neuropati
1. Polineuropati : lesi mengenai banyak saraf tepi dengan pola distribusinya bilateral simetris dan kejadiannya boleh dikatakan simultan. Kadang-kadang dipergunakan istilah poliradikulopati, poliradikuloneuropati untuk menekankan keterlibatan radix spinalis ataupun radix spinalis plus truncus spinalis.
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Kategori Klinis Neuropati
2. Mononeuropati dan mononeuropati multipleks : lesi mengenai satu saraf tepi, atau beberapa saraf tepi yang sifatnya tidak simetris. Atau dapat juga nampak simetris, tetapi kejadiannya tidak simultan . Dari patologinya juga akan nampak berbeda, seringkali tergantung dari lokasi masing-masing saraf yang terkena.Etiologi kategori ini umumnya adalah penyakit ataupun kondisi yang menimbulkan lesi atau kerusakan yang sifatnya local, bukan difus seperti pada polineuropati (trauma atau mikrotrauma mekanik : traksi, tekanan, pukulan langsung, luka tembus; entrapment, lesi karena radiasi, listrik, terbakar, lesi vaskuler, lesi granulomatus, lesi neoplastik).
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• Beberapa neuropati dengan nyeri yang menonjol diantaranya adalah : neuropati sensorik diabetika, iskemia, alkohol, malnutrisi
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NYERI NEUROPATIK
Nyeri yang didahului lesi primer atau
disfungsi pada sistim saraf perifer
maupun sentral
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Allodinia : Nyeri yang disebabkan oleh stimulus yang secara normal tidak menimbulkan nyeri
Disestesia : Sensasi yang tidak menyenangkan, baik bersifat spontan maupun dengan pencetus
Anastesia Dolorosa : Nyeri pada area yang seharusnya bersifat anastetik
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KAUSALGIA
Nyeri seperti terbakar, Alodinia, Hiperpatia yang menetap seringkali bercampur
dengan disfungsi vasomotor, Sudomotor, dan Gangguan Tropik.
(CRPS – II = Complex Regional Pain Syndrome)
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INJURY
TISSUE:
INFLAMMATION OUTCOME
NERVE
5. Neuropathy
6. Axotomy
REVERSIBLE1. Brief2. Moderate
1,5 10115015 100+
Minutes Days
REGENERATION
TISSUE LOSS
HEALING
PERSISTENT DAMAGE
Byers & Bonica, 2001
4. Chronic Inflammation
IIREVERSIBLE3. Severe
OR
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KERUSAKAN JARINGAN
INFLAMASI
SSA
MI
NOS
SENSITISASI
AKTIFASI
ECT. DISC.
Si-Na+
KORNU DORSALIS
PgB5HTAdenosin
PengalamanKognitifBehaviourPsikologik
Inhibisi desenden
OTAK
PAIN – NO PAIN
R-NE
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Polyneuropathy is caused by the degeneration of axon terminals and results in symmetric distal sensory loss with shading to normal sensation. A compression neuropathy often results in demyelination with the axon left relatively intact. Sensory loss follows a radicular pattern. When the neuronal cell body dies the condition is called "neuronopathy." If the cell body is in the sensory ganglion the condition is often referred to as "ganglionopathy." The pattern is usually random
Axonal polyneuropathyDemyelinating NeuropathyNeuronopathy (Ganglionopathy)
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BIOMEDIKAL(BIOMEDICAL)
BIOPSIKOSOSIAL(BIOPSYCHOSOCIAL)
NOSISEPSI(NOCICEPTION)
NYERI(PAIN)
PENDERITAAN(SUFFERING)
PERILAKU NYERI(PAIN BEHAVIOUR)
PENGERTIAN MODEL NYERI
BYERS AND BONICA, 2001MODIFIKASI PENULIS
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Anger
Fear
Anxiety
Depression
Noxious Stimuli
PS
YC
HO
LO
GIC
AL
NOCICEPTIVE
A
B
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EPIDEMIOLOGI
• Nyeri Neuropati : 20 % di klinik nyeri
• Nyeri Neuropati : 2 – 40 % dari nyeri kronik
• Nyeri Neuropati :
- Neuropati Diabetes 60,4 %
- Polineuropati distal 47,3 %
- Herpes Zoster 4,8 promil
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KESULITAN MENENTUKAN PREVALENSI
1. Sindrom nyeri neuropatik beraneka-ragam
2. Respon terhadap terapi berbeda-beda
3. Prosedur diagnostik perlu teliti dan cermat
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ETIOLOGI (Pokdi PERDOSSI 2000)
1. Saraf tepi :a. Trauma : Neuropati jebakan, Transeksi saraf
termasuk pembedahan, kausalgia, amputasi dan nyeri tungkai, nyeri perut, nyeri pasca torakotomi
b. Mononeuropati : Diabetik, Invasi nervus/ pleksus oleh keganasan, Iradiasi pleksus, Iradiasi iskemik, Penyakit jaringan ikat (artritis rematoid, SLE, Poliartritis nodosa)
c. Polineuropati : Diabetik, alkohol, Nutrisi, Amiloid, Penyakir Fabry, Neuropati, Isoniasid, Idiopatik
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2. Radik dan ganglion : diskus prolaps, arachnoiditis, avulsi radiks,Rizotomi operatif, neuralgia pasca Herpes, Neuralgia Trigeminus, Kompresi Tumor
3. Medula Spinalis : Transeksi total, Hemiseksi, Kontusio, Kompresio, Hematomieli, Pembedahan, Siringomieli, Sklerosis Multiple, AVM, Disrafisma, Defisiensi Vit B12 , Mielitis Sypilis.
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4. Batang otak : Sindroma Wallenberg,
Tumor, Siringobulbi, Sklerosis Multipel,
Tuberkoloma.
5. Talamus : Infark, Hemorargik, Tumor,
Lesi bedah pada nukleus sensorik
6. Korteks/subkorteks : Infark, AVM,
Trauma, Tumor
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ETIOLOGI1. Trauma : - Sindrom jebakan
- Complex regional pain syndrome- Amputasi- Lesi Medula Spinalis- Operasi
2. Infeksi : - Herpes Zoster- AIDS- Tabes Dorsalis- Lepra
3. Tumor : - Kompresi- Infiltrasi- Paraneoplastik- Metastatis
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ETIOLOGI4. Vasculer :- Lupus Eritematosus
- Artritis Rematoid- Stroke
5. Toxin : - Obat sitostatika- Arsen, Plumbum, Magnesium
6. Defisiensi Nutrisi :- Vitamin B1, B6
- Alkohol Neuropati7. Genetik :
- Penyakit Fabry
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ACUTE FLAXID PARALYSISACUTE FLAXID PARALYSIS
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Deteksi dini kasus AFP
1.Jangan menganggap ringan keluhan flu-like symptom (anak belum imunisasi)
2.Waspada bila disertai keluhan kelemahan anggota gerak
3.Tindak lanjut pada keluhan flu-like simptom pada daerah yang ditemukan kasus AFP
4.Jangan memanipulasi pasien-pasien yang dicurigai (point 1-2)
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Kasus AFP di Rumah Sakit
• Semua kasus secara klinis memperlihatkan kelemahan yang bersifat lower motor neuron (flaksid) yang timbul secara mendadak (akut).
- kurang dari 2 minggu
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Tabel 1. Penyebab Kelemahan Akut
• Sel Kornu Anterior- Poliomielitis
- Penyakit motor neuron
• Saraf- Sindroma Guillain Barre
- Tick Paralisis
- Difteri
- Keracunan Logam Berat
• Paut Saraf Otot- Miastenia Grafis
- Miastenia yang diinduksi Obat
- Sindroma Miastenik
(Eaton- Lambert)
- Keracunan Organophosphat
• Otot- Polimiositis
- Periodik Paralisis
- Miopati Toxic
- Mioglobinuria/Rhabdomiolisis
- Sindroma Neuroleptic Maligna
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Gambar potongan melintang Medula Spinalis
Radik dorsalis saraf spinal
Neuron Motorik
Radik ventralis saraf spinal
T Sel ganglion (neuron sensorik)
Saraf Spinal
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Kelumpuhan LMN
• Akibat langsung hilangnya fungsi kerusak-an sel kornu anterior atau akson dari radik anterior dan saraf tanda dan gejala sesuai lokasi lesi.
• Disertai gangguan sensibilitas : Terlibatnya saraf campuran (motorik dan sensorik) atau mengenai kornu anterior dan posterior
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Gejala kelumpuhan
LMN UMN• Sifat Flaksid Spastik• Reflek
Fisiologis / ( - ) Patologis ( - ) ( + )
• Tonus • Trofi Atrofi ( + ) Atrofi ( - ) /
Disuse atrofi
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Syndroma Guillain Barre (SGB)Acute Inflammatory Demyelinating Polineuropathy (AIDP)
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Syndroma Guillain Barre (SGB)Acute Inflammatory Demyelinating Polineuropathy (AIDP)
• Kelainan sistim saraf akut-difus pada radix spinalis, saraf perifer dan kadang-kadang saraf kranial
• Timbul setelah suatu infeksi• Penyakit Autoimune• 0,6 - 1,9 orang / 100.000 /tahun• Terdapat di seluruh dunia, setiap musim• Menyerang semua umur• Tersering dewas muda
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Gambaran Klinis
1.Kelumpuhan :
- Simetris, tipe LMN
- Sebagian besar diawali kedua ekstremitas
bawah secara asenden badan,
anggota gerak atas, saraf Kranial
- Otot proksimal lebih berat dari pada
distal atau sebaliknya, atau sama beratnya
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2. Gangguan sensibilitas
Defisit sensoris obyektif minimal
Sering distribusi pola kaus kaki-sarung tangan
3. Saraf kranialis
N. VII kelumpuhan otot-otot wajah
N. III, VI diplopia (melihat kembar)
N. IX, X sulit menelan, sengau
4. Gangguan fungsi otonom
- Takikardi - Bradikardi
- Hipertensi - Hipotensi
- Retensio urine
5. Kegagalan pernafasan : paralisis diafragma dan otot-otot pernafasan. 10 - 33 % penderita
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Perjalanan penyakit
Fase
Progresif
Fase
Plateau
Fase
Penyembuhan
Kelumpuhan
Bbrp Hari - 4 mg
2 Hari - 3 mg Bbrp Bulan Waktu
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Pemeriksaan
• LCS : Peninggian kadar protein tanpa diikuti peninggian jumlah sel
dissosiasi sito-albumin
• EMG : Pelambatan pada kecepatan hantaran gelombang F (segmen proksimal dan radiks saraf)
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DUCHENNE MUSCULAR DYSTROPHY
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DUCHENNE MUSCULAR DYSTROPHY
merupakan distrofi otot yang relatif sering dijumpai dan sifatnya progresif.
Insiden di luar negeri berkisar antara 150-250 per 1000.000 kelahiran hidup
Dijumpai pada semua ras.
Diturunkan secara resesif, sex-linked (X) dan dominan pada laki-laki, dengan umur awitan antara 2-4 tahun.
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• Umumnya baru diketahui setelah anak mulai berjalan, dimana pada perkembangannya anak nampak lamban dan mudah jatuh.
• Gejala pertama yang timbul adalah kelemahan otot gelang panggul, yang berakibat munculnya waddling gait dan Gower’s sign. Pada tanda Gower nampak sebagai berikut: bila anak ditelentangkan di lantai, ia akan cenderung memanjat diri sendiri (climbing up himself) untuk mencapai posisi tegak. ( Gambar 1)
• Lengan akan terkena kemudian, sedangkan otot muka terlibat paling akhir.
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Gambar 1. Gower’s sign
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• Sekitar 80% menunjukkan pseudohipertrofi otot, utamanya otot betis. Tampak terlihat otot betis, (gastrocnemius) dan lengan (triceps dan deltoideus)
• Sebaliknya otot gelang bahu dan panggul, serta otot paraspinal mengecil. Keterlibatan otot yang sakit umumnya simetris.
• Tampak juga posisi lordosis lumbal yang progresif.
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• Refleks tendo menurun sampai menghilang, karena otot tidak memberikan reaksi kontraksi.
• Karena saraf masih baik, maka tidak ada gangguan sensibilitas.
• Pada stadium akhir, otot diafragma dan interkostal dapat menjadi lemah sehingga akan mengganggu sistem pernapasan. Kapasitas pernapasan menurun sehingga rentan terhadap infeksi paru seperti pneumonia.
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Pemeriksaan penunjang
• aldolase,
• CPK (creatine phosphokinase),
• SGOT (serum glutamicoxalacetic transaminase),
• SGPT (serum glutamicpyruvic transaminase)
• lactic dehydrogenase
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• pemeriksaan elektromiografi (EMG) : konduksi saraf normal.
• Pemeriksaan serabut otot dengan jarum menunjukkan gambaran khas miopati yaitu motor unit potensial (MUP) ampli-tudonya kecil, durasinya pendek, sebagian polifasik
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Diagnosis
1. mengetahui awitan kelemahan otot, 2. pseudohipertrofi, 3. karakteristik distribusi kelemahan otot, 4. riwayat keluarga, 5. kenaikan kadar ensim serum, 6. dan biopsi otot.
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Pengobatan
• Belum ada terapi yang efektif untuk DMP• diharapkan suatu terapi gen. • Pasien diusahakan untuk selalu aktif selama dan
sedapat mungkin. • Program fisioterapi direkomendasikan secara reguler. • Bila kontraktur dapat dirujuk ke bagian bedah ortoped.
Penggunaan ortose ringan atau brace untuk stabilisasi pergelangan kaki atau sendi yang lain dan mencegah deformitas
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MYASTHENIA GRAVIS
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MYASTHENIA
• An acquired autoimmune disorder causing skeletal muscle fatigue and weakness
• Autoantibodies against the acetylcholine receptor produce weakness that can affect the entire body or only eye movement
• Can begin at any time, from early childhood to extreme old age
• The cause of the autoantibodies is not known. The thymus is implicated in the inception and generation of the autoantibodies
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PENDAHULUAN• tidak terdapat gangguan pola gerak, sebab yang
terganggu adalah kekuatan otot yang semakin berkurang apabila menjalankan aktifitas, kekuatan otot akan pulih kembali setelah beristirahat
• Di negara maju prevalensinya 1 : 10. 000 – 50.000 penduduk, dengan frek tertinggi pada kelompok umur 20 – 30 tahun, walaupun jarang dapat pula menyerang neonatus, anak anak, orang tua
• wanita mempunyai resiko dua kali lebih besar dibanding dengan pria.
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Etiopatogenesis
• Akibat reaksi immunologi (pembentukan antibodi)
– atropi membran reseptor post sinaptik– Celah antara lipatan membran menjadi lebih lebar
• Kholinesterase mempunyai waktu untuk menguraikan
acethycholin
Menjadi cholin & acetat
• Acethylcholin yg sampai pd membran post sinaptik tdk
mencukupi untuk depolarisasi
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Myasthenia gravis junction AChR reduced; synaptic fold simpified; synaptic space widened; nerve terminal normal
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PATHOLOGY/PATHOPHYSIOLOGY
_______________________________________
� 3 Mechanisms by which autoantibodies to
ACh receptors interfere with NM transmissions:
Compliment
Modulation of ACh receptors
Direct block
_______________________________________
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PATHOLOGY/PATHOPHYSIOLOGY■ Compliment Mediated lysis • Presence of IgG on postsynaptic membrane together with lytic component of compliment■ Modulation of ACh receptors Rate of degeneration increases after they have
been crosslinked by antibodies. • Average lifespan of a normal ACh receptor is
7 days • For a myasthenic patient, it is 1 day■ Direct block Antibodies directly inhibit receptor function
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Normal AChR
1 2
3
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Auto antibodies bind to the acetylcholine receptor and cause increased receptor degradation. The combination of the binding and the turnover effects results loss of receptor so that an action potential in the motor neuron does not always result in an action potential in the muscle fiber
Thymoma as present in some patients with myasthenia
Onset in non-thymoma cases:
Peak incidence at 10-30 yeas of age, again at 60-70 yeas of age
Myasthenia associated with thymoma:
Peak incidence at 40-50 years of age
Under 40 predominantly affects women
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MYASTHENIA WHICH HAS A DIFFERENT MECHANISM
• Neonatal myastheniaTransient illness, lasting less than 1 month, 1 in 8 babies of myasthenic mothers
• Juvenile myastheniaMyasthenia in the younger age group, generally similar to those of myasthenia in young adults
• Penicillamine-induced myastheniaUsually resolves over several month after drug withdrawal
• Lambert-Eaton myasthenic syndromeA presynaptic disorder characterized by impaired release of Ach from the nerve terminal.
60% cases is associated with small cell lung carcinoma
• Congenital myasthenia• Familial myasthenia
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SYMPTOMS AND SIGNSweakness of skeletal muscle is
characteristically increased by exercise, but is not associated with muscle pain (in contrast to ‘physiological” fatigue)
emotional stress, pregnancy and infection can also cause an exacerbation of symptoms
• Ocular muscles• Limb weakness
• Bulbar muscle weakness• Respiratory muscle involvement
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CLINICAL CLASSIFICATION
Main groups of acquired myasthenia gravis
Group I: ocular myasthenia gravis (symptoms may remain persistently confined to the ocular muscles, particularly when 2 years have elapsed since the onset
Group IIA, B: mild or moderately severe generalized myasthenia gravis
Group III: acute severe (fulminating) myasthenia gravis with respiratory muscle involvement
Group IV: late (chronic) severe disease
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INVESTIGATION
• Anti-ACHR antibody• Antistriated muacle antibody
• Edrophonium chloride test
• Electromyographic techniques
• Thymoma
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MODES OF THERAPY
• Anticholinesterase therapy: – Pyridostigmine, 30-120 mg orally– Neostigmine bromide, 15-30 mg orally every 3 hours except at
night
Higher dose than those given above are seldom indicated and greatly increase the risk of choinergic crisis.
Side-effects are caused by para sympatithetic stimulation and include: - pupillary constriction
- colic- diarrhoea- Increased salivation- Increased sweating- Increased lacrimation- Increased bronchial secretions
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MODES OF THERAPY
• Corticosteroids:Prednisolone - suitable
- once daily on alternate days to avoid side-effects- initial dose 10 mg, increased slowly
out patients: 5-10mg / week in patients: 5-10 mg / dose
to avoid the exacerbation of symptoms that can occur when the drug is started at a high dose
- Maximal dose: 1-1.5 mg / kg body weight - (or symptoms are controlled)
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Thymectomy
Intravenous immunoglobuln
Plasma exchange
• Improvement is expected although most patients are maintained on a low-dose corticosteroid after their initial tapering
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MYASTHENIC CRISIS
■ Muscle weakness is due to an insufficient amount of ACh.■ Performing a Tensilon test will yield an immediate improvement in muscular contractions.■ Signs and symptoms: Increased pulse rate Absence of cough or gag reflexes Positive response to Tensilon test
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MYASTHENIC CRISIS
occurs with inadequate treatment and can be precipitated by infection.
Treatment consist of:
• Control of the airway and assisted ventilation• Anticholinesterase medication• Immunosuppressive drug therapy and/or plasma
exchange
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CHOLINERGIC CRISIS■ Muscle weakness is due to excess of ACh at motor effector sites.■ Results from an overdosing of anticholin- esterase medications■ Signs and symptoms Negative response to Tensilon test Nausea and vomiting Bradycardia and hypotension Fasciculations Diplopia Diarrhea and abdominal cramps
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CHOLINERGIC CRISIS
caused by excess anticholinesterase medication
Treatment consist of:
• Control of the airway and assisted ventilation• Temporary withdrawal of anticholinesterase drugs,
with later reintroduction at a reduced dose regiment• Immunosuppressive drug therapy and/or plasma
exchange
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Polio Mielitis Anterior Akut
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Polio Mielitis Anterior Akut
Patofisiologi • Polio virus : jalur Fecal - Oral infeksi primer pada TGI
(individu tidak imunisasi)• Replikasi virus : Faring - TGI
Masa inkubasi 1 - 3 minggu Virus penetrasi dinding intestinum, darah seluruh bagian tubuh
• Sistim saraf hanya sebagian kecil dari pasien• 95 % : - Infeksi asimtomatik atau
- Penyakit tidak spesifik “carrier”
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Keadaan tertentu
• Viremia sistem saraf pusat secara selektif ; pada kelompok neuron khususnya sistim motorik
• Terberat pada sel-sel kornu anterior medula spinalis segmen lumbal “Poliomielitis Paralitikus” cz terkena tungkaig’ bs jalan
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Gambaran klinik
• Sebagian besar infeksi ; asimtomatis• Gejala sistemik ringan : Faringitis - gastro-enteritis,
flu - like symptom abortif poliomielitis• Perkembangan kearah kelemahan
- Diawali dgn : Panas, Lesu, Nyeri kepala, mual.
- kaku leher dan punggung
Penyakit dapat berhenti pada tahap ini tanpa
komplikasi sebagai : “Aseptic meningitis”
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• Berkembang dengan kelemahan.
• Otot sensitif, kaku & nyeri .
• Kelemahan asimetris dengan bagian proksimal lebih berat dari distal.
• Tungkai lebih sering dari lengan
• Fase akut : Keluhan parestesi, secara obyektif tidak ada.
• Refleks tendo menurun s/d menghilang
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• Tonus otot menurun Atrofi berkembang setelah 5 - 7 hari dari onset nyata setelah 3 minggu
• Distribusi kelumpuhan bervariasi :- Bayi/anak <5 tahun : terbanyak asimetri,
umumnya 1 tungkai.- Anak lebih tua : 1 atau 2 tungkai.- 16 - 65 thn : asimetri pada ke 4 anggota gerak.
• 10-15% mengenai otot bulber : nukleus N.IX-X kelemahan otot pharing dan laring.• Keadaan berat gangguan pernapasan dan menelan problem kardio respirasi.
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Post Polio Syndrome
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Post Polio Syndrome
• Pada individu-individu 20 - 50 th setelah infeksi primer.
• Kelemahan dan atrofi : berkembang secara lambat - bertahap.
• Problem : - gangguan respiratorik
- skoliosis
• Tidak ada bukti terdapatnya virus polio
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Diagnosis
• Poliomielitis anterior akut bila perkembangan akut kelemahan tipe flaksid yang asimetris, tanpa gangguan sensibilitas
• Perubahan LCS : Pleositosis
• Secara pasti : isolasi virus polio dari : tenggorokan, tinja atau autopsi medula spinalis
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Terapi
• Tidak ada terapi spesifik
• Simtomatis penting
Fase akut rawat inap Rumah Sakit
• Analgetik, kompres hangat untuk nyeri otot
• Pengawasan tanda-tanda komplikasi respiratorik
• Fisioterapi dan alat-alat bantu
• Cukup istirahat / hindari kelelahan
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PARALYTIC POLIO
Persons infected with polio: > 95% asymptomatic viremia and spontaneous clearing
Flulike prodrome severe generalized myalgias with focal,
often asymmetric fasciculation;
followed by weakness that often is severe
the legs often are most affected
although any muscle or region can be
involved including diaphragm and bulbar
muscles
Recovery typically is incomplete, atrophy and asymmetric weakness is often permanent
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POST-POLIO SYNDROME
• Occasionally a syndrome develops in former paralytic polio victims several years following the initial attack
• Patients typically complain a diffuse myalgias and recurrence of weakness n muscles that were affected in the initial attack
• The lag between the initial attack and development of so-called post-polio syndrome often is measured in decades
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SELESAI