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PENDAHULUAN
POLINEURITIS AKUT PASCA INFEKSI, POLINEURITIS AKUT TOKSIK, POLINEURITIS FEBRIL, POLIRADIKULOPATI / ACUTE ASCENDING PARALYSIS. DEFINISI : SINDROM INI DICIRIKAN OLEH KELUMPUHAN / KELEMAHAN OTOT EKSTREMITAS YANG AKUT DAN PROGRESIF, BIASANYA MUNCUL SETELAH INFEKSI INSIDENSI RATA RATA PERTAHUN 1 - 2 / 100. 000 POPUlASI PEREMPUAN : LAKI LAKI = 2 : 1
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Georges Guillain
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T lymphocytes
CD8- recognize epitopes paired c MHC-I CD4- activate and control the immune response Scavenger cells break down antigen into small peptide fragments (T cell epitopes), MHC-II epitope complexes are expressed on the surface & the scavenger become an APC which docks on a CD4 c a compatible TCR. CD4 proliferates releasing cytokines.
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Antibodies
Cytokines activate other lymphocytes including B cells that differentiate into plasma cells and serve as immunoglobulin factories. Abs are Ig molecules that recognize, bind, neutralize and opsonize Ag for phagocytosis. They activate complement(membrane attack complex) & induce target cells to activate the inflammatory response
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Self-tolerance
The process of self recognition T & B cells learn self tolerance during maturation Autoimmunity occurs when the mechanisms of self protection are defective
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Mechanisms of Autoimmunity
Molecular mimicry- microbe cell surface Ag resembles self protein. Damage results from friendly fire The inciting Ag is usually unidentified & may not exist as a single stimulus. Excessive cytokine release due to profound immune stimulus may awaken self tolerant T cells or may cause expression of MHC complexes. Self Ags bound to drugs may lose tolerated status
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PATOLOGI
REAKSI INFLAMASI (INFILTRAT) DAN EDEMA SARAF YANG TERGANGGU SEL INFILTRAT TERUTAMA SEL LIMFOSIT DAN TAMPAK PULA MAKROFAG DAN PMN PADA PERMULAAN PENYAKIT SETELAH ITU TIMBUL SEL PLASMA DAN SEL MAST SERABUT SARAF MENGALAMI DEGENERASI SEGMENTAL DAN AKSONAL
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TYPE OF GBS
AIDP CIDP AMSAN AMAN MILLER FISHER SYNDROM Acute Panautonomic Neuropathy
Features of AIDP
2/3s have identifiable preceding event 50% begin with paresthesias followed by weakness in legs; 10% begin with arm weakness; rarely begins in face Ophthalmoplegia: partial 15%, total 5% Autonomic dysfunction in 65%, arrhythmias, hypotension,urinary retention in 10-15%, pupillary inequality
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AIDP
Progresses for days to 4 weeks 15% with severe disability Mortality 3-5% CSF: protein may be normal early, elevated in 90% by clinical nadir, cells< 10 in 95%, >50 suggests HIV EDX: prolonged F & distal motor latencies, conduction block 30-40% in routine studies
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AIDP
Pathology: immune attack directed at schwann cell plasmalemma esp. at nerve roots with IgG & complement deposits preceding demyelination
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CIDP
Evolves over months Fluctuates Respiratory failure, dysautonomia, facial weakness, ophthalmoplegia- all are rare CSF protein often highly elevated Marked slowing of motor nerve conduction Steroid responsive
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Features of AMSAN
Commonly preceded by diarrhea esp. c. jejuni Abrupt onset of weakness c rapid progression to quadriplegia & respiratory insufficiency Other features as c AIDP Longer recovery, more residual & mortality 10-15%
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AMSAN
CSF as in AIDP EDX: no response in some motor nerves, decreased amplitude of the CMAPs, fibrillations on needle study, absent SNAPs Immune attack directed at axon plasmalemma at nodes of Ranvier. Wallerian degeneration
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Features of AMAN
Often preceded by diarrhea affecting younger population in China. Sporadic in USA Prognosis similair to AIDP Mortality <5% EDX: reduced CMAPs c normal F & distal motor latencies and sensory studies. Fibrillations in 2-3 weeks
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AMAN
Pathology: again axonal plasmalemma at nodes of Ranvier sometimes limited to physiologic dysfunction c nodal lengthening. May go on to extension through axonal basal lamina. Most axons recover s Wallerian degeneration
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Differential Diagnosis
Consider the possibility of an upper motor neuron lesion Other considerations are rare. Diphtheritic neuritis & poliomyelitis belong more to the history section of this presentation. A new possibility is West Nile Virus.
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Differential
N-M: MG, LES, Antibiotics Toxic: Cigutera (ciguatoxin), Pufferfish (tetrodotoxin), Shellfish (saxitioxin), Botulism, Tick paralysis (Lone Star tick, Gulf Coast tick), Glue sniffing, Buckthorn Mononeuritis multiplex assoc. c Wegners. PAN, SLE, RA, Sjogrens, Cryoglobulinemia etc.
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Differential
Metabolic: Periodic paralyses, Hypokalemia, Hypermagnesemia, Hypophoshatemia c parenteral hyperailimentation, Thyrotoxicosis, ICU myoneuropathy (CIP) Heavy metal: Lead, Arsenic, Thallium, Barium c hypokalemia
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Treatment
Respiratory failure Autonomic dysfunction DVT & PE Pain Positioning & Skin care Physical therapy Nutrition
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Respiratory Failure
Oropharyngeal weakness in ~25% with impaired swallowing of secretions & aspiration Mechanical respiratory failure- mainly due to diaphragmatic weakness (Phrenic nerves.) Inspiratory c MIF (Max. Inspir. Force) a good supplement measure to FVC
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Respiratory Failure
~33% require intubation Avg. time to intubation is 1 week & these pts. have substantially longer recovery time Need is unlikely if patient does well for 2 wks. post onset of paresthesiaes Guidelines: FVC <15 mL/kgm MIF < 25 cm water
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Psychological
Fear Helplessness Communication Pain Sleep deprivation & hallucinosis Depression Visits from other GBS patients
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Personal Experience
Bowes, Denise; The doctor as patient: an encounter with Guillain-Barre syndrome. Can Med Assoc J 131:1343-1348
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Corticosteroids
Lancet 1993 242 pts. IV Methylprednisilone 500 mgm/day x 5. Ineffective May cause relapse
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Plasma Exchange
Removal of the bloods liquid soluble components including complement, immunoglobulin, immune complexes, cytokines and interleukins A typical session removes about 60% of the body mass of plasma proteins which is replaced c saline, albumin & FFP Done qod for 3-5 sessions
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Plasma Exchange
Various studies since 1985 Time on ventilator reduced by Full strength regained at 1 year: Exchange 71%, Untreated 52% Limitations: Limited availability Avoid with autonomic instability
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IVIG
Mechanism of action- unknown ? Antiidiotypic antibody action ? Inhibition of cytokines ? Sponging of complement ? Binding to Fc receptors so macrophages cant bind
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IVIG
Dosage: 0.4 gms/kgm/day x 5 c each dose given over 3-4 hours preceded by IV diphenhydramine &/or po ibuprofen Caution c renal insufficiency or IgA deficiency 38 Center trial in 1997 Equal to plasma exchange
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J.H.C. 48 yo welder
June 02 H.A. followed in 2 wks by Lt. Facial weakness June 03 Rhinorrhea & cough August 6 Pain lt. Hip spreading over a few days to back 7 legs August 15 Legs buckle c lt.facial weakness 1 wk later. LP c protein of 70. NCVs c prolonged F waves
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1 Week post discharge, elevated titers to West Nile Virus Follow up at 1 month- continued improvement
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ETIOLOGI
INFEKSI VIRUS (DULU) KELAINAN IMUNOLOGIK
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ETIOLOGI
PENYEBAB INFEKSI VIRUS (KELOMPOK HERPES SERING CYTOMEGALOVIRUS ATAU EPSTEIN BARR
VIRUS).
BAKTERI (CAMPYLOBACTER JEJUNI, MYCOPLASMA PNEUMONIAE).
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GAMBARAN KLINIK
TANDA DAN GEJALA KELEMAHAN MOTORIK TERJADI AKUT (PARAPARESIS LMN). KELEMAHAN TERJADI, 50 % MENJELANG 2 MGG, 80% MENJELANG 3 MGG > 90 % MENJELANG 4 MGG
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GAMBARAN KLINIK
KELEMAHAN / KELUMPUHAN SIMETRIS (PARAPARESIS / PLEGIA LMN ATAU TETRAPARESIS / PLEGIA LMN) KASUS RINGAN HANYA TERBATAS KEDUA TUNGKAI KASUS BERAT TERJADI TETRAPLEGIA LMN DENGAN CEPAT DALAM WAKTU < 72 JAM ASCENDING LANDRYS PARALYSIS.
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GAMBARAN KLINIK
HIPOTONI DAN HIPOREFLEKSI SELALU DITEMUKAN. GGN SENSORIK RINGAN, PROPRIOSEPTIF NORMAL
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GAMBARAN KLINIK
PROSES PENYEMBUHAN 2 4 MINGGU TERHENTINYA PROGRESIVITAS KLINIK, NAMUN DPT TERTUNDA SELAMA 4 BLN. KLINIS, PENDERITA SEMBUH FUNGSIONAL NAMUN PEMERIKSAAN EMNG MASIH MENUNJUKKAN KELAINAN
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LABORATORIUM
DARAH TEPI : NORMAL ATAU LEUKOSITOSIS LIKUOR SEREBROSPINALIS : 48 JAM S/D AKHIR MGG I DISSOSIASI SEL ALBUMIN (ALBUMINO CYTOLOGIC DISSOCIATION ~ SEL NORMAL, PROTEIN SANGAT TINGGI (DPT S/D1000 MG%), PUNCAKNYA PADA MINGGU KE 4 6. ~ REAKSI INFLAMASI ENMG (ELEKTRONEUROMIOGRAFI) :HARI I / II, DIULANG 1 MGG DEMIELINISASI : KHST AMPLITUDO NORMAL DEGENERASI AXONAL : KHST , AMPLITUDO
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TERAPI
TIDAK ADA DRUG OF CHOICE WASPADAI KLINIK MEMBERAT (GGN OTOT- OTOT PERNAPASAN) RAWAT DI ICU MONITORING VITAL CAPACITY ( 15 ML/KGBB) INTUBASI TRACHEAL INTAKE TERJAMIN, ADA GGN MENELAN PASANG NASOGASTRICTUBE R/ KORTIKOSTEROID KONTROVERSIAL
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TERAPI
R/ SPESIFIK : (BAIK KASUS AKUT)
PLASMAEXCHANGE : HARI 1, 3, 5 DAN 7 .
PEMBERIAN CUKUP 2X PADA KASUS RINGAN DAN 4 X PADA KASUS SEDANG DAN BERAT DOSIS : 50 ML/ KGBB TARGET VOLUME EXCHANGE
IMMUNOGLOBULIN DOSIS TINGGI (IV) : SETIAP HARI DOSIS : 2 GR/ KGBB DIBAGI DALAM 5 DOSIS (400 MG/KGBB/DOSIS)
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TERAPI
R/ ROBORANSIA SARAF FISIOTERAPI HARI I / II SETELAH MRS (PASIF), TAHAP PEMULIHAN (AKTIF)
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PROGNOSIS
BAIK USIA MUDA TIDAK MEMERLUKAN PERNAPASAN BUATAN ONSET LAMBAT TIDAK TEJADI KELUMPUHAN TOTAL PENYEMBUHAN BERVARIASI BEBERAPA MINGGU S/D BULAN
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