Sgb Retno Ms
Transcript of Sgb Retno Ms
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GUILLAN-BARRE SYNDROME
(GBS) LANDRYS ASCENDING PARALYSIS,
ACUTE IDIOPATHIC POLYRADICULITIS
is an inflammatory disorder of the peripheralnerves.
Is characterized by weakness and numbness ortingling in the legs and arms, and possible loss ofmovement and feeling in the legs, arms, upper
body, and face.
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Guillain-Barre Syndrome
Synonyms Acute inflammatory demyelinating
polyradiculoneuropathy-AIDP Landry-Guillain-Barr syndrome,
Landry-Guillain-Barr-Strohl syndrome,
Acute idiopathic neuropathy,
Acute demyelinating neuropathy,
Infectious polyneuritis,
Acute polyradiculoneuritis,
Axonal Guillain-Barr syndrome, Acute motor axonal neuropathy,AMAN,
acute motor-sensory axonal neuropathy-AMSAN
Miller-Fisher syndrome,
Pharyngeal-cervical-brachial GBS
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Variants with rapid progressive phase:
AIDP(Acute Inflammatory Demyelinating
Polyradiculoneuropathy) = GBS
AMSAN (Acute Motor Sensory Axonal
Neuropathy
AMAN (Acute Motor Axonal Neuropathy)
MFS (Miller-Fisher Syndrome)Also known asMiller Fish syndrome, Miller's syndrome and
Acute Disseminated Encephalo-
myeloradiculopathy
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Variants with slow progressive phase:
CIDP (Chronic Inflammatory
Demyelinating Polyneuropathy)
MMN (Multifocal Motor Neuropathy
MMSD (Multifocal Motor SensoryDemyelinating Neuropathy
MADSAN (Multifocal Acquired
Demyelinating Sensory AcquiredNeuropathy
PDN (Paraproteinaemic Demyelinating
Neuropathy
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AIDP (Acute Inflammatory Demyelinating
Polyradiculoneuropathy) = GBS
About 80% of the patients get this form.
- AMSAN (Acute Motor Sensory AxonalNeuropathy
A serious axonal form of GBS that attacks
motor and sensory nerves. Fulminantcourse with slow and incomplete
recovery.
AMAN (Acute Motor Axonal Neuropathy) particularly severe form, attacks the motornerves primarily, causing rapid progressiveweakness often with respiratory failure. Pure
axonal cases may occur more frequently in
other parts of the world outside Europe and
North America. AMAN cases also may be
different from cases of axonal GBS describedin the West.
Many cases have been reported in rural areas
of China, especially in children and young
adults during the summer months.
Prognosis is often quite favorable and
recovery is rapid.
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Incidence
In developed countries, Guillain-Barre
Syndrome (GBS) is the most common causeof acute neuromuscular paralysis.
about 1 to 2 cases in every 100,000 people
per year ( 0,01%-0,02% of populations) in the United States afflicting about 5,000
persons annually
Age:All ages, Bimodal distribution, withpeaks in age ranges of 15-35 years and 50-
75 years
Sex:Male-to-female ratio is 1.5:1
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Incidence variant SGB(race)
demyelinating
polyneuropathy
Europe
Africa
Axonal degeneration
Axonal degenerationAsia
South America
demyelinating
polyneuropathy
North America
demyelinatingpolyneuropathy
demyelinating
polyneuropathy
Australia
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Causes autoimmune disease triggered by a preceding
bacterial or viral infection. Campylobacter jejuni,cytomegalovirus, Epstein-Barr virus andMycoplasma pneumoniae Chlamydia HepatitisB,mononucleosis, AIDS, and herpes simplex.
Sometimes Guillain-Barre occurs following
surgery or vaccinations (such as rabies and swineflu vaccines) or in association systemic lupuserythematosus orHodgkin's disease.
are commonly identified antecedent pathogens
A small number of casesto occur after a medicalprocedure, such as minor surgery.
Camden, NJ. Review provided by VeriMed Healthcare Network. Update
Date: 4/25/2004
http://www.nlm.nih.gov/medlineplus/ency/article/000594.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001324.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000580.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000580.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000435.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/001324.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000594.htm -
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Causes
GBS is a postinfectious immune-mediated diseasecellular and humoral immune mechanisms
Most patients report an infectious illness in the weeks priorto the onset of GBS.
Guillain-Barr syndrome may be an autoimmunedisorder in which the body produces antibodies thatdamage the myelin sheath that surrounds peripheral nerves.
The myelin sheath is a fatty substance that surroundsaxons. It increases the speed at which signals travel alongthe nerves.
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INFECTION COMMON INSIDEN = 13 - 72% SEVERE MOTOR
AKSONAL NEUROPHATY
Ab GM1
C jejuniHadden, 2001
INF C jejuni PREDICT PROGNOSIS severe inflammation 0f axonal
disruption and loss.
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PATOG : SEL T, MAGR, SITOKINE
MIP-1 a
MCP-1
EAN
Sitokine gerakleuk & macr & ek
MIP-1 a dan MIC-1Gold-2000, Kiesier-2000,
Fujioka-99
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Cytokines
Macrophage
TNF
IL-1
IL-1raIL-6
IL-12
IFN-gTNF, IL-6 IL-1, IL-1ra IL-12
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Histologic Findings
Lymphocyte and macrophage infiltration ofperipheral nerves. Macrophage influx
multifocal demyelination .
in severe inflammatory changesCellular
infiltratesWallerian degeneration
cranial nerves, nerve roots, dorsal root
ganglions, and peripheral nerves.
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GENE TRANSCRIPTION
NF-kBmRNA proteinmacrofag
Immun mediator
Damage peripheral nerve
1. Ekspression NF-kB correlationswith macropagh
2. Magr imun reactions Andorfer, 2001Jander, 2001
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Pathophysiology 4:
APOPTOSIS SEL Th1 Apoptosis genetic proggramed cell death Apop sel T cell (auto reactive) healing
(Gold et al., 1999).
Apop Schwann cell remyelinization nerveregenerations
Th1 TNF-a Apoptosis Schwann cell (Weishaupt et al., 2001).
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APOPTOSIS
Auto reactive
myelin-directed T cell
imun response-inflamation
Gold, 1999
Schwann cell
Remyelinisation +
Nerve regeneration Weishaupt, 2001
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Pathophysiology 5:
molecular mimicry
Campylobacter jejuniantecedent illness andGBS can be typified by infections.
The virulence presence of specific antigens in
its capsule. Immune responses ( against the capsular
components) produce antibodiesreact withmyelin demyelination.
Ganglioside GM1cross-react with C jejunilipopolysaccharide antigens immunologicdamage to the peripheral nervous system.
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INFECTION _ NEUROPHATY
MOLECULAR MIMICRYCONCEPT
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MOLECULAR MIMICRY CONCEPT
C jejuni
H. Influ
C jejuni sialicacid synthesaGM1,GD1, GT1 (Yuki, 2001)
Anti-GT1 IgG post infection H Infl
(Koga, 2
C jejunigene cst II activity(transkrsialic acid) (Van Belkum, 2001)
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Signs and Symptoms
The first symptoms: numbness or tingling(paresthesia) in the toes and fingers ( Gloveand stocking ) progressive weakness inthe arms and legs over the next few days.
Some paresthesia only in their toes and legs
Others only experience symptoms on oneside of the body.
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________________% of patient
Sensory symptoms
numbness 72pain 37
Sensory sign 62
Reflexes
normal 5
partial loss 17
complete loss 78
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__________________% 0f patient
urinary dysfunction 25
rectal disfunction 14 hypotension 14
hypertension 31
sinus tachycardia 36 Arrhythmia 16
hyponatremia (SIADH) 9
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Pain 50% described the pain severe and distressing due to direct
nerve injury or the paralysis and prolonged immobilization
inflamed nerve roots.back and leg pain as aching or throbbing, myalgic with
cramping, local muscle tenderness, visceral pain, and painassociated with conditions of immobility (eg, pressure nerve
palsies, decubitus ulcers).
Dysesthetic symptoms 50% of patients frequently are described asburning, tingling, or shocklike sensationsmore prevalent in thelower extremities persist indefinitely in 5-10% of patients.
Intensity of pain on admission correlates poorly with neurologicdisability on admission and end outcome.
C i l i l
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Cranial nerve involvement
Cranial nerve involvement 45-75%
Common complaints : Diplopias,Dysarthria,Facial droop, Dysphagia
Facial and oropharyngeal weakness usually
appears after the trunk and limbs areaffected.
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Weakness
Ascending and symmetricaldevelops acutely andprogresses over days to weeks mild weakness tocomplete tetraplegia with ventilatory failure.
Peak deficits are reached by 4 weeks . Recoveryusually begins 2-4 weeks after progression ceases.
The lower limbs usually are involved before theupper limbs. Proximal muscles may be involvedearlier and more severe than the distal onesTrunk,bulbar, and respiratory muscles can be affected.
Diagnosis
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Diagnosis The diagnosis of Guillain-Barr syndrome is based on
typical clinical features, electrodiagnostic examination,
and examination of the cerebrospinal fluid. An EMG (a test of electrical activity in muscles) showslack of nervous stimulation.
A CSF (cerebrospinal fluid) examination may beabnormal, showing increase in protein without increase in
white blood cell count. Electromyogram (EMG)diagnostic tool recordsmuscle activity, the loss of reflexes (slowing of nerveresponses. )
Nerve conduction velocity (NCV) records the speed
at which signals travel along the nerves. AnNCV (nerveconduction velocity) shows demyelination.
Lumbar puncture : An elevated level of protein in thefluid is characteristic of GBS.
Diagnostic Criteria for Typical Guillain-Barr
http://www.nlm.nih.gov/medlineplus/ency/article/003929.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003625.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003927.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003927.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003625.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003929.htm -
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Diagnostic Criteria for Typical Guillain-Barr
Syndrome Features required for diagnosis
Progressive weakness in both arms and legs
Areflexia Features strongly supporting diagnosis
Progression of symptoms over days, up to four weeks
Relative symmetry of symptoms
Mild sensory symptoms or signs
Cranial nerve involvement, especially bilateral weakness of facialmuscles
Recovery beginning two to four weeks after progression ceases
Autonomic dysfunction
Absence of fever at onset
High concentration of protein in cerebrospinal fluid, with fewer
than 10 cells per cubic millimeter Typical electrodiagnostic features
Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barr syndrome. Ann Neurol 1990;27(suppl):S21-4.
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GBS EMERGENCY
SEVERE &ACUT PARALYSIS
Severe Weakness
BULBAR PARALISIS
RESP Failure
OTONOMIC Complications
MORTALITY (Hahn, 1998). (Raphael et al, 2000).
Respiratory involvement
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Respiratory involvement
40% of patients have respiratory and oropharyngeal weakness.
Typical complaints may include the following:
Dyspnea on exertion
Shortness of breath
Difficulty swallowing Slurred speech
Ventilatory failure required respiratory support in up to one
third of patients .
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Guillain Barre Disability Score
(Greenwood, Osterman, GBSG studies)
1=minor symptoms
2=able to walk 5 meters without assistance
3=able to walk 5 meters with assistance
4=confined to bed or chair5=requiring
assisted ventilation for at least part of the
day or night
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MANAGEMENT
Early intensive care
R) spesifik against otoantibody &reaktivitas el imunokomp IVIg (dosis & plasmaph(PE)
(Hartung et al., 2002).
R) General supportif & Complications
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GENERAL1. Respiratoy failure : Intubation; volume control
ventilation; antibiotics; glycopyrrolate (forcontrolling secretion & diarrhea).
2. Nutrition3. Prophylaxis against deep vein thrombosis and
pulmonary embolism4. Stress ulcer prophylaxis5. Prevention of decubitus ulcers6. Keeping patients head 30-45% above the bed7. Others (treat hypokalemia; hypophosphatemia; HCt 30-40/menit.
Tracheostomi bulbar palsy (aspiration) (Sharshar et al, 2003).
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ASSESSMENT OF SYMPTOMS FORINTUBATION AND MECHANICALVENTILATION (Leshner 1984)
SYMPTOM RESULT__________________________________________________________
___ Vital capacity
3 times predicted tidal volume Poor cough
2 times predicted tidal volume Intubation indicated Maximum respiratory forces
PE max 40 cm H2O Inability to clear secretionsPI max 20 cm H2O Marked weakness inspiratory
muscles Dysphagia with bulbar paralysis Danger of aspiration Arterial blood gases:
Hypoxia with normal PCO2 Probable severe ventilatoryimpairment
____________________________________________________________
PE = max. pressure on expiration; PI = max. pressure on inspiration
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Postponing intubation may lead to so-called "crash"intubation in the middle of the night which may haveadditional complications.
Time on the ventilator varies but most modern intensivecare series report a median of 30 days.
A tracheostomy is soon considered. In the vast majority ofpatients, this is usually postponed until
the third week after intubation,
bulbar symptoms or in thosesevere damage to the axonstracheostomy considered earlier
IVIg
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IVIg
Indication : severe paralysis, resp failure, bulbar
Dose = 0.4 g /kgBW/day (5 days)
2g Efektive 2 first week
R) gagal = 25%
trial Ig &prednisolon
Side effect (10%) headache (respon NSAID),myialgia,
aseptic meningitis(5%), Self limiting 24-48 hours
Profilak100mg Solu-Cortef IV anafilaksis (def IgA) rare (Raphael et al, 2000).
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High dose prednisone and imuran again put
me in remission, this time for two years. In
May 1995, I was started on IV-IG (80g) andimuran (175mg). The imuran did nothing
and was stopped after a year. In 1997, I was
put on low dose
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PLASMAPHERESIS = PE
CHANGE humoral immunity ventil time , motoris , squele Efektive < 7 days
Indication:paralisis prog, bulbar/resp
Dose = 200-250 ml/bw (5 X/10-14days)
albumin low Na 5%
Compl phlebitis & hypotension (Raphael et al, 2000).
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Complications:
Breathing difficulty (respiratory failure)
aspiration of food/fluids into the lungs pneumonia
increased risk of infections
deep vein thrombosis permanent loss of movement of an area
contractures of joints or other deformity
Complications
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Complications
Monitor heart rate, blood pressure, and cardiacarrhythmias allows early detection of life-threatening situations.
Antihypertensives and vasoactive drugs patientswith autonomic instability.
Enteric or parental feedings for patients onmechanical ventilation and severe dysphagia
to ensure that adequate caloric needs are met whenthe metabolic demand is high.
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Complications
Bowel and bladder dysfunction Initial
managementsafe evacuation and
prevention of overdistension.
Monitoring for secondary infections, such
as a urinary tract infection.
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Mortality/Morbidity:
In epidemiologic surveys, the overall death
rates range from 2-12% of patients.
GBS-associated mortality rates increasemarkedly with age. Though the death rate
increases with age
Males have a death rate 1.3 times greaterthan females after the age of 40 years.
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